Topical Steroids Withdrawal (TSW)

Topical corticosteroid withdrawal is an infrequent rebound reaction that happens in patients who have excessively used topical steroids and occurs following cessation.

This condition is typically a result of long-term application of topical corticosteroids with moderate to high potency. Withdrawal symptoms might manifest as skin burning, itching, redness, scaling, swelling, papules, or pustules.

Etiology:

The etiology of topical steroid withdrawal may be explained by the following mechanisms:

  • Tachyphylaxis, which is a decrease in response, occurs with continued application, resulting in the need for higher doses.
  • Patients with atopic dermatitis who do not respond well to topical steroids have an upregulation of glucocorticoid receptor b.
  • Following the withdrawal of topical steroids, keratinocytes continue to suppress the formation of self-cortisol.
  • Topical corticosteroids induce vasoconstriction, however, upon discontinuation, there is a rebound effect of vasodilation due to an increase in the release of nitric oxide, resulting in skin redness (erythema).
  • Disruption of the barrier leads to a subsequent release of cytokines, triggering a cascade of immune responses, after the anti-inflammatory effects of topical corticosteroids are stopped.

Clinical manifestations:

The withdrawal symptoms might begin anytime from 48 hours to more than 3 months after discontinuation. 

Withdrawal generally manifests in four distinct stages:

  • Typically, a few days after stopping the treatment, there is a sudden eruption of intensely red and oozing skin, which may spread to places that were not previously treated.
  • The skin experiences dryness and itchiness accompanied by shedding, also known as desquamation.
  • The skin begins to regenerate, although it becomes more sensitive and may experience occasional flare-ups.
  • The skin returns to the state that it was before topical corticosteroid cessation. The duration of the recovery process can range from several weeks to several years.

Symptoms: 

  • Severe itch
  • Intense itching
  • Burning pain 
  • Insomnia
  • Depressed state
  • skin hypersensitivity
  • Intolerance to emollients.

Skin characteristics:

  • Erythematous skin
  • Elephant wrinkles refer to the thicker skin with decreased flexibility that typically affects the extensor surfaces of the body.
  • The red sleeve sign refers to the presence of erythema (redness) on the limbs, but the palms and soles are not affected.
  • The headlight sign is characterized by redness (erythema) across the face, but specifically excluding the nose and the area around the mouth (perioral skin).
  • Skin shedding (desquamation)
  • Edema
  • Exudate.
  • Telangiectasia
  • Papules with or without nodules
  • Pustules. 

Diagnosis:

One of the challenges is determining whether the observed skin reaction is a result of discontinuing topical corticosteroids or a deterioration of the underlying skin condition for which the steroids were given.

The diagnostic criteria for topical corticosteroid withdrawal lack consensus. However, recent literature highlights the following key features: 

  • Frequent and prolonged topical steroid use on the area of initial eruption
  • Frequent topical steroids  use on the face or genital area
  • Sensation of burning or itching

Often associated with:

  • Atopy history, particularly atopic dermatitis
  • History of using oral prednisone to treat skin conditions
  • sensitivity 
  • Skin shedding 
  • Swelling, particularly in the eyelids or ankles, and the presence of ‘elephant wrinkles’ on the back of the elbows and front of the knees (extensors).
  • Red sleeve sign.

Patch testing is a valuable tool for ruling out contact dermatitis caused by topical corticosteroids, cream excipients, or other topical treatments such as emollients. However, this can be challenging if there is not enough healthy skin available for testing.

Histology is not a reliable method for diagnosing topical corticosteroid withdrawal because the histological signs it presents, such as epidermal atrophy, spongiosis, and parakeratosis, are not specific enough.

Management:

General measures:

  • Emollients and moisturizers.
  • cold compresses, the use of ice, and the administration of gabapentin for burning pain.
  • Antihistamines are used to alleviate itching.
  • Pain management with simple analgesic.

Specific measures: 

  • The primary treatment approach is discontinuing the use of topical corticosteroids, while closely monitoring for any rebound reactions. 
  • There is controversy around tapering vs abrupt withdrawal.
  • Gradually reducing the dosage of oral corticosteroids.
  • Dupilumab should be taken into consideration for people with atopic dermatitis.
  • Prevention and treatment of any subsequent infection.
  • Phototherapy.
  • Immunosuppressants. 

Prevention:

  • Gradually decrease the frequency and potency of topical corticosteroids once the inflammatory skin conditions have resolved.
  • Avoid prolonged use of strong topical corticosteroids on the face.
  • Reduce the length of continuous and prolonged corticosteroid treatment, for example, to less than 2 weeks. Certain disorders, including vulval lichen sclerosus, may require a therapy duration beyond 4 weeks in order to optimize the favorable response.
  • Decrease the strength and frequency of topical steroid usage from daily to twice weekly after 2-4 weeks of treatment.

Finally, atopic dermatitis and other skin disorders should be well treated despite concerns about the risk of topical corticosteroid withdrawal, since a considerably higher proportion of patients react to effective topical steroid use than have withdrawal symptoms.

Written by:

Mashael Alanazi, Medical student

Revised by:

Maee Barakeh, Medical student

References:

DermNet

Paraneoplastic Pemphigus

Paraneoplastic pemphigus (PNP) is an uncommon autoimmune blistering mucocutaneous illness that is nearly invariably linked to an undetected or diagnosed tumor. In 3–5% of cases, it is the most severe but least common form of pemphigus. PNP can affect toddlers as well, even though it often affects adults between the ages of 45 and 70 who have an equal preference for male and female sexes. Nearly all PNP instances have a malignant condition beneath them.

Causes: 

Although the exact origin of PNP is unknown, it seems that an underlying malignancy, most frequently a lymphoproliferative tumor, triggers an immunological reaction. The primary autoantigenic target in PNP is desmosomes. Possible causes and their underlying mechanisms a generated autoreactive T-cell induced humoral and cell-mediated immunity, IgG1 autoantibodies against desmosome and hemidesmosome, and specific gene variants, or alleles, such as the HLA-Cw*14 allele and the HLA Class II DRB1*03 allele.

Clinical features:

Often the only presenting feature of PNP is extensive refractory painful mucous membrane blisters, and hemorrhagic crusting involving the mucous membrane of various organs including the oropharynx, conjunctiva, lips, and genitals to name a few. A pruritic diffuse polymorphic rash can be seen following mucous membrane involvement. Systemic affection includes muscle weakness, and internal organs can be affected as well, with signs and symptoms of hidden malignancy. 

Diagnosis:

A combination of clinical and histological findings, immunological serology, direct and indirect immunofluorescence, and other methods are used to diagnose PNP while keeping in mind that a search for underlying malignancy is important. 

Histology findings depend on the clinical phenotype, the findings vary from suprabasal acantholysis, and lichenoid interface changes with a dense mononuclear immune cell infiltrate in the dermo-epidermal junction.

Direct immunofluorescence reveals granular or linear IgG and C3 deposits along the basement membrane zone at the dermo-epidermal junction. While indirect immunofluorescence reveals circulating antibodies targeting the intercellular proteins of cells within stratified squamous or transitional epithelium are characteristic of PNP. To diagnose PNP, autoantibodies against the 190-kD band of periplakin and the 210-kD band of envoplakin can be found. This method is both specific and sensitive.

Management:

The treatment of PNP is difficult and requires a multidisciplinary approach. General measures for PNP include an identification of infection, dehydration, extensive wound care, and analgesic mouthwashes. The best course of treatment for PNP is debatable, and response to therapy varies. Possible courses of treatment include topical corticosteroids or topical calcineurin inhibitors, Systemic immunosuppression agents, IVIG, and plasmapheresis. It will be necessary to treat the underlying malignancy with appropriate chemotherapy, radiation, or immunotherapy, or by surgical excision. It may take 6-18 months to observe complete resolution of the lesions after benign neoplasm excision.    

Complications: 

The complications of PNP include secondary bacterial infection, dehydration, ocular complications (pseudomembranous conjunctivitis, and, progressive scarring), and lung complications (bronchiolitis obliterans, and respiratory failure).

Prevention:  

Prevention is difficult because PNP is usually always linked to underlying disease, especially hematopoietic cancers like leukemia and lymphoma. For PNP patients, surgical removal of solid tumors combined with high-dose IVIG used both before and after surgery can reduce the incidence of bronchiolitis obliterans.

Outcome:  

PNP has a fatality rate that can range from 70% to 90%. The prognosis appears to be poorer for PNP patients with the erythema multiforme-like subtype. Widespread infections, the advancement of cancer, or bronchiolitis obliterans are among the causes of death. The prognosis for PNP may be improved by treatment using a range of medications.

Written by:

Mohammed Alahmadi, Medical Student. 

Revised by:

Maee Barakeh, Medical Student. 

References:

DermNet

Dermatology by Bolognia

Medscape

Cutis Verticis Gyrata

What is Cutis verticis gyrate?

 Cutis verticis gyrata is an uncommon, congenital, or acquired scalp disorder characterized by an overgrowth of the scalp skin resulting in convoluted folds and deep furrows that mimic the surface of the cerebral cortex. It is more prevalent in males than females. The majority of primary cases develop after puberty and usually occur before the age of thirty. Some secondary types of cutis verticis gyrata may be present at birth.

 

Cause of Cutis verticis gyrata

Cutis verticis gyrata is caused by the proliferation of the scalp skin. It is categorized as:

  • Primary essential cutis verticis gyrata with no other related abnormalities. Primary indicates that the etiology of the disease is unknown.
  • Primary nonessential cutis verticis gyrata is linked to neuropsychiatric illnesses such as epilepsy, seizures, cerebral palsy, and ophthalmologic abnormalities.
  • Secondary cutis verticis gyrata can be caused by many disorders that alter scalp structure, such as:
  1. Acromegaly
  2. Melanocytic naevi (moles)
  3. Birthmarks, including connective tissue naevus , fibromas and naevus lipomatosus
  4.  Inflammatory conditions include eczema, darier disease, psoriasis, folliculitis, impetigo, atopic dermatitis, and acne. 

 

Clinical features of Cutis verticis gyrata

Cutis verticis gyrata usually affects the center and back of the scalp, although in certain cases it can affect the entire scalp. The folds feel soft and spongy. It cannot be rectified by pressure. The skin color is unaffected. The number of folds might range from two to over ten. Folds in primary cutis verticis gyrata are usually symmetric, while those in secondary cutis verticis gyrata are asymmetric.

 

Diagnosis of Cutis verticis gyrata

Cutis verticis gyrata is typically a clinical diagnosis.
Investigations for neurological diseases or underlying disorders may involve:

  • skin biopsies
  • radiography, such as MRI.

 

Treatment of Cutis verticis gyrata

Cutis verticis gyrata therapy includes maintaining adequate scalp hygiene to prevent secretions from accumulating in the scalp’s furrows. For cosmetic reasons, definitive surgical treatment may be requested. Secondary cutis verticis gyrata is treated based on the underlying condition.

 

Prognosis of Cutis verticis gyrata

Cutis verticis gyrata is a progressive condition. It is mostly bothersome because of its cosmetic appearance. It is rarely complicated by melanoma growing within a melanocytic naevus.

 

 

Written by:

Atheer Alhuthaili , Medical Student. 

Revised by:

Maee Barakeh, Medical Student

References:

DermNet

UpToDate

Medscape

Stasis Dermatitis (Venous Eczema)

Definition:

Venous dermatitis is a common type of eczema/dermatitis that involves one or both lower legs in association with venous insufficiency. It is also known as gravitational dermatitis.

Epidemiology:

Venous eczema primarily occurs in middle-aged and elderly individuals, with a prevalence of 20% in those over 70 years old. It is related with:

  • Stasis ulcers.
  • History of deep venous thrombosis in an affected limb
  • History of cellulitis 
  • Chronic edema in the lower leg, exacerbated by high temperatures and long periods of standing
  • Varicose veins

Etiology:

Venous dermatitis is caused by fluid accumulation in the tissues and triggering of the innate immune response.

Typically, during walking, the leg muscles facilitate the upward flow of blood and valves in the veins prevent blood from accumulating. Deep venous thrombosis (DVT) or varicose veins may compromise the valves in the deep leg veins. Back pressure causes fluid accumulation in the tissues. An inflammatory reaction occurs.

Clinical manifestation:

The condition usually manifests as red, scaly, and eczematous patches or plaques on chronically edematous leg. The medial ankle is most commonly and severely affected, with skin changes possibly extending to the knee and foot. Pruritus can lead to lichenification due to continuous scratching or rubbing.

Chronic forms are characterized by scaling and hyperpigmentation from cutaneous hemosiderin accumulation. Additional signs of chronic venous insufficiency and associated comorbidities are frequently observed, such as:

  • Atrophie blanche —refers to stellate, porcelain-white scarring patches caused by microthromboses. 
  • The lower leg shaped like “Champagne bottle” — narrowing at the ankles and induration (lipodermatosclerosis)
  • Ulceration

Complications:

  • Impetiginisation — is a secondary infection caused by Staphylococcus aureus that leads to the formation of yellowish crusts.
  • Cellulitis — an infection caused by Streptococcus pyogenes, characterized by redness, swelling, pain, fever, a red streak up the leg, and swollen lymph nodes in the groin.
  • Secondary eczema — spread of eczema to other parts of the body.
  • allergic reaction to one or more ingredients in the lotions or ointments used

Diagnosis:

Stasis dermatitis is usually diagnosed clinically on the basis of:

  • Skin lesions’ clinical presentation 
  • Venous insufficiency history
  • Other chronic venous insufficiency manifestations (e.g., varicose veins, pitting edema, hyperpigmentation) 

A skin biopsy is usually unnecessary to diagnose stasis dermatitis. If the diagnosis is unclear or if the patient presents with unusual characteristics (such as a single lesion or no ankle involvement), performing a biopsy and histopathologic examination can help confirm the diagnosis and rule out other skin conditions like allergic contact dermatitis, asteatotic eczema, or cutaneous malignancies. 

Management:

General measures:

  • Lifestyle modification— Patients with chronic venous disease and stasis dermatitis should make lifestyle adjustments to lower venous hypertension, decrease edema, and prevent venous ulcers. These consist of weight loss, regular walks, avoiding extended standing, exercising, and elevating your legs whenever possible. 
  • Skin care — To treat skin dryness and itching in mild or chronic stasis dermatitis, it is recommended to use mild, fragrance-free liquid cleansers for gentle skin washing and apply bland emollients regularly. White petroleum jelly is effective, affordable, and non-irritating. Emollients can be administered frequently throughout the day.

Compression therapy: 

Compression therapy using either compression bandaging systems or compression stockings, is the primary conservative treatment for stasis dermatitis and the associated chronic venous disease.

 

 

Written by:

Mashael Alanazi, Medical Student.

Revised by:

Maee Barakeh, Medical Student.

Resources:

DermNet.

Uptodate.

Pityriasis Alba

Pityriasis alba is a skin condition that primarily affects people between the ages of three and sixteen in children and young adults. It is common worldwide with a prevalence in children of around 5%. It affects males and girls female equally. Pityriasis alba hypopigmentation is more noticeable and possibly more prevalent in black skin types than in white skin types.

Etiology: 

The etiology of pityriasis alba is idiopathic. It is usually associated with atopic dermatitis and dry skin. It frequently appears after sun exposure, maybe because the afflicted spots become more noticeable due to surrounding skin darkening. Ongoing research about the relationship between pityriasis alba and the following factors: UV radiation, insufficient or excessive bathing, low levels of copper, and Malassezia yeasts.

Clinical features:

The patients will usually present with 0.5 to 2 cm multiple hypopigmented patches or thin plaques on the cheek and chin. The patches could be oval, round, or irregular in shape with poorly defined edges. Usually, the patients will have absent or minimal itch. The dryness and scaling are more noticeable in the winter, while the hypopigmentation is more noticeable in summer.

Pityriasis alba patches undergo several stages:

  1. The appearance of scaly pink with just a palpable surface.
  2. The hypopigmentation stage with fine surface scale.
  3. The post-inflammatory hypopigmented macule/ patch with no scale.
  4. Resolution of the lesion. 

Diagnosis:

The diagnosis is usually clinical, and the investigations are done to rule out other differential diagnosis:

  • Wood lamp: No enhancement in the cases of pityriasis alba.
  • Scrapings for mycology: negative in the cases of pityriasis alba.
  • Skin biopsy: Usually not indicated, would reveal spongiotic dermatitis and melanin reduction.

A dermoscopy of pityriasis alba will reveal, a poorly defined pale area, white structureless areas, superficial white scale, and normal hair color. 

Management:

Asymptomatic pityriasis alba requires no treatment. A moisturizing cream to improve the dryness, and a mild topical steroids will reduce redness and itchiness if present. It has been observed that calcineurin inhibitors, such as tacrolimus ointment and pimecrolimus cream, speed up the return of skin color and maybe just as effective as hydrocortisone.

Psoralen + UVA photochemotherapy is used in severe situations, this treatment may aid in repigmentation; nevertheless, the likelihood of recurrence following treatment discontinuation is considerable. Laser therapy can be effectively treated with a twice-weekly, 308-nm excimer laser treatment for 12 weeks. 

Prevention:  

Sunscreen use could be utilized to reduce the development of pityriasis alba.

Outcome: 

Pityriasis alba usually goes away in a year, although it might take anything from a few months to two or three years. The color gradually reverts to its original state.

Written by:

Mohammed Alahmadi, Medical Student. 

Revised by:

Maee Barakeh, Medical Student. 

References:

DermNet.

Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology.

Dermatology by Bolognia.

Erythema Dyschromicum Perstans ( Achy Dermatosis )

Definition:

It is a type of acquired macular hyperpigmentation that occurs on the face, neck, and trunk. It is characterized by distinct circular or irregular patches that have a gray color. Erythema dyschromicum perstans is also known as ashy dermatosis (of Ramirez) due to its color. 

Epidemiology:

The disease typically manifests in the second or third decade of life, with no predilection for a particular gender. Lesions, however, can develop in old people or very young children.

Etiology:

The precise etiology of erythema dyschromicum perstans remains unknown. Due to its histological characteristics, it is frequently categorized as a form of lichen planus pigmentosus. There are some theories including:

-Genetic predisposition

-Allergic reaction to cosmetics and hair dye

-Viral infections 

-An adverse effect of drugs and medications

Clinical features:

-The pigmented patches might be either symmetrical or unilateral in their distribution.

-Early lesions may have an elevated appearance, a more distinct border, and a reddish color. This stage is not necessarily noticeable.

-Other than that, the patient is well and has no related illnesses or abnormal blood tests.

Diagnosis:

Occasionally, the clinical presentation of erythema dyschromicum perstans may exhibit sufficient characteristic features to provide a diagnosis of the condition. A skin biopsy can show mild vacuolar degeneration of the basal layer in initial lesions, and pigmentary incontinence with dermal melanophages in more developed patches.

Management:

Erythema dyschromicum perstans shows a high level of resistance to the treatments currently available. Although it may remain unchanged for years, some cases ultimately resolve on their own.

Possible interventions to enhance appearance of erythema dyschromicum perstans include the following: 

-Topical corticosteroids

-Ultraviolet radiation exposure 

-Pigment lasers, such as the Q-switched ruby laser

-Chemical peels

Clofazimine has proven to be the most effective systemic therapy. Dapsone, griseofulvin, hydroxychloroquine, isoniazid, and corticosteroids have demonstrated efficacy in a few cases.



Written by:

Mashael Alanazi, medical student.

Resources:

DernNet.
Bolognia Textbook of Dermatology.

Image resource: Bolognia Textbook of Dermatology.

Tinea Versicolor

What is Tinea Versicolor?

Tinea versicolor is a commonly occurring, benign, superficial cutaneous fungal infection that is typically characterized by patches and macules on the back and chest that are either hyper- or hypopigmented. It is caused by Malassezia species. Despite the availability of efficient oral and topical treatment methods, disease recurrence is frequent.

 

Epidemiology of Tinea Versicolor

Tinea versicolor occurs worldwide, with a higher occurrence in tropical climates. Adolescents and young adults are the most common age groups to develop tinea versicolor. It can also impact children and infants.

 

Risk Factors for Tinea Versicolor

There are various risk factors associated with this disease. These risk factors include exposure to hot and humid weather, hyperhidrosis, use of topical skin oils, genetic predisposition, immunosuppression state, malnutrition, corticosteroid usage, and Cushing disease. It may clear in the winter and reoccur each summer.

 

Cause of Tinea Versicolor

Tinea versicolor does not result from a dermatophyte infection. It is considered non-contagious. The causative organism is a Malassezia species. It is a component of the normal skin flora, but it can cause the disease when it transforms into its pathogenic hyphal form. The reasons for the transformation of tinea versicolor are likely to be multifactorial.

 

Clinical Features of Tinea Versicolor

Tinea versicolor is typically asymptomatic; some complain of mild pruritus, with the main issue being its appearance.Tinea versicolor lesions are characterized by hyperpigmented, hypopigmented, or erythematous patches, macules, or thin plaques with a mild scale. To demonstrate this scale, the skin surface may need to be scratched. These lesions are usually small (<15 mm) and range in color from coppery brown, paler than the surrounding skin, or pink. The most typical areas of involvement are the upper trunk and proximal upper extremities. Less frequently, faces (common in children) or intertriginous areas are affected.

 

Diagnosis of Tinea Versicolor

Tinea versicolor is usually diagnosed clinically. Dermatoscopy and Wood’s lamp examination may show signs suggestive of tinea versicolor but do not confirm the diagnosis. Dermoscopic examination reveals fine-scale, nonuniform pigmentation within lesions and perilesional hyperpigmentation or hypopigmentation. Wood’s lamp examination exhibits yellow to yellow-green fluorescence in certain patients. The KOH preparation findings in tinea versicolor are considered diagnostic. It shows hyphae and yeast cells that resemble spaghetti and meatballs.

 

Management of Tinea Versicolor

The management of Tinea versicolor can be summarized into a few fundamental aspects:

1:Patient education

2: Topical antifungals, which are considered first-line therapy, including Selenium sulfide, zinc pyrithione, azoles (miconazole or ketoconazole), or allylamines (terbinafine)

3: Oral antifungals are only for infections that are severe, extensive, or resistant to topical treatment (not commonly used in children). Oral itraconazole and fluconazole are the recommended oral medications.

 

Recurrence of Tinea Versicolor

Tinea versicolor has a high recurrence rate. Preventive treatment with topical or oral medication administered intermittently is required to avoid recurrences in most cases.

 

 

Written by:

Atheer Alhuthaili , medical student. 

Revised by:

Maee Barakeh, medical student.

References:

Bolognia textbook of dermatology.

DermNet.

UpToDate.

Medscape .

Harlequin Ichthyosis 

A severe genetic variant of ichthyosis called harlequin ichthyosis manifests at birth as thicker, rigid skin plates that cover the entire body like armor. The condition is uncommon and equally affects men and women. Approximately one in 300,000 neonates are affected by it. Harlequin ichthyosis is not known to be racially predisposed. There could be a higher occurrence in societies where paternal consanguinity is prevalent.

 

Causes:

Harlequin ichthyosis is inherited in an autosomal recessive manner. The ABCA 12 gene alterations are to blame. It is thought that the ABCA12 gene encodes a transporter protein that is necessary for healthy skin development and is involved in the movement of epidermal lipids across cell membranes. The cell cannot produce any ABCA12 protein due to certain mutations in the ABCA12 gene. Additional mutations result in the synthesis of an atypically tiny protein that is incapable of carrying lipids in an appropriate manner. Harlequin ichthyosis is characterized by hard, thick scales that are caused by a disruption in the normal development of the epidermis caused by a loss of functional ABCA12 protein.

 

Clinical features:

The clinical features include skin changes present at birth, significantly thickened, with deep erythematous fissures separating the scales and huge, glossy plates of hyperkeratotic scale. Facial features include degraded nasal alae and blocked nares. The external auditory canal may be blocked by scale, and the ears’ pinnae and retroauricular folds may be tiny or nonexistent. The lips’ traction results in eclabium.

 

Diagnosis:

Genetic laboratory testing and a physical examination of the patient are necessary for the diagnosis of harlequin ichthyosis. The most precise diagnostic procedure for harlequin ichthyosis involves looking for a loss of function mutation in the ABCA12 gene using genetic testing. Gene mutations may result in smaller versions of the proteins essential for skin development as well as poor lipid transport in the skin. A collodion membrane and congenital ichthyosiform erythroderma-like appearance are the outcomes of less severe mutations. Histology of the skin biopsy reveals hypergranulosis, parakeratosis, and a significantly thicker stratum corneum.

 

Management:

Treatment for harlequin ichthyosis focuses on skin protection and infection control; there is no known cure. For several weeks during their early years, the majority of harlequin babies require one-on-one nursing care. Infection prevention or treatment during this period may also require antibiotics. After bathing, when the skin is still damp, softening emollients especially those that contain urea, salicylic acid, or alpha hydroxy acids work exceptionally well. These products help to maintain the skin’s suppleness and moisture content while guarding against the fissures and cracks that can result in a subsequent bacterial infection. It has also been demonstrated that early systemic treatment with oral retinoids (such as acitretin or isotretinoin) improves overall survival, softens, or resolves plate-like scales, and heals skin fissures.

 

Complications: 

Poor immunity, infections, muscle shortening, limb necrosis and amputation, and respiratory failure.

 

Outcome:  

Infants with harlequin ichthyosis in the past hardly made it past the first few days of life. The survival rate has increased due to innovations in newborn care; globally, rates are getting close to 50%.  Youngsters who make it through the neonatal stage typically acquire a less severe form of ichthyosis, but they still exhibit fish-scale scales, seborrheic patches that retain waxy, yellowish material, generalized poor hair growth, and scarring alopecia.  Those who survive harlequin ichthyosis may nevertheless experience hypothyroidism, small stature, arthralgias, digit contractures, and failure to flourish.   

 

Written by:

Mohammed Alahmadi, Medical Student. 

Revised by:

Maee Barakeh, Medical Student. 

References:

DermNet

Dermatology by Bolognia

Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology

Image source: Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology 

 

ORF

Orf is a skin infection caused by parapox virus, a zoonotic contracted from goats and sheep. Because the condition causes crusts and scabs around the mouth, nose, teats, and other areas, it is also known as contagious pustular dermatitis. The disease lasts for roughly six weeks, and it is extremely contagious.

 

Cause and Mode of Transmission: 

Direct inoculation of contaminated material, frequently through an already-existing wound or scratch, results in lesions in humans. Shearers, freezer workers, sheep producers, and anyone bottle-feeding a lamb run the danger of contracting orf. Youngsters may potentially get it from playing in a sheep shed or on contaminated pasture. There have been reports of human orf following unintentional contact with animal-targeted orf virus vaccination. It is not typically spread from person to person, however sporadic reports of this have occurred.

 

Clinical Features:

The incubation period lasts from 5-6 days. Patients usually present with a 2-3 cm solitary small, firm, red lump that enlarges to form a flat-topped, blood-tinged pustule or blister on the forearms, hands, and fingers. Large lesions could be observed in immunocompromised individuals. 

Typically, making an incision through the skin will reveal firm, red tissue beneath, despite the appearance of pus from the skin. In the early stages, the orf lesion is frequently sensitive and irritating. Lymphangitis and lymphadenopathy of the inner side of the elbow and/or under the arm are not uncommon. These features could be associated with mild fever.  

 

Diagnosis:

A clinical diagnosis of orf is typically made in a person who has experience working with sheep or goats. PCR can be used to confirm the infection in vesicular fluid, skin biopsies, or viral swabs. Electron microscopy can be used to identify it as well. 

 

Management:

Most of the time, no special care is required because orf resolves on its own in roughly six weeks. While it is extremely rare, the lesion should be covered to avoid infecting the surrounding area or other persons. Shave excision is one method of removing large lesions. Antibiotics are the appropriate treatment for secondary bacterial infections. A few cases of orf have been found to respond well to imiquimod cream.

 

Complications: 

The complications of orf include secondary bacterial infection, erythema multiforme, toxic eythemas, and rarely pemphigoid.

 

Prevention:  

Farmers who raise sheep and goats should be mindful of the risk of orf and handle the animals especially lambs and young while using non-porous rubber gloves with washing hands. It is advised to vaccinate the herd using a live virus in the event of a severe orf outbreak.

 

 

Written by:

Mohammed Alahmadi, Medical Student. 

Revised by:

Maee Barakeh, Medical Student. 

References:

DermNet.

Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology.

Andrews’ Diseases of the Skin: Clinical Dermatology.

Nodular Prurigo

Nodular prurigo (NP), also known as prurigo nodularis, is a chronic dermatological disorder characterized by intensely pruritic, firm nodules. Although it can form anywhere on the body, a symmetrical distribution of arms, legs, back, and torso is where it most frequently appears.

 

Etiology:

It is still unclear what specifically causes NP. Based on skin biopsies, it is believed to be related to a dysregulation of the nerves, with more nerve cells seen in the dermis and fewer in the epidermis. 

There is also an association with atopy, certain drugs, and systemic conditions such as chronic kidney disease, liver disease, diabetes, and malignancy.

 

Clinical features:

The severity of NP can range from a few to hundreds of lesions of different sizes.

At first, the rash:

– Include tiny, red, or pink bumps typically 3–8 mm in diameter.

– Has a hyperpigmented border

– Very itchy 

– Is distributed in readily accessible body regions, including the lateral aspects of the arms and legs, shoulders, chest, and buttocks.

Sometimes, it begins as a papule and progresses to a nodule or plaque.

Scratching results in:

-Enlargement producing a raised, warty, nodular surface. The nodules have a symmetrical distribution and are usually hard and small, but they can reach up to 3 cm in diameter.

-Ulceration of the skin that can lead to an increased risk of infection.

-Healed lesions may leave a scar or discoloration.

 

Diagnosis:

The diagnosis of NP is clinical, based upon the clinical presentation of distinctive excoriated, nodular lesions frequently distributed symmetrically and a patient’s history of persistent, intense pruritus. A skin biopsy is not routinely performed to confirm the diagnosis.

It is essential to evaluate systemic causes of persistent pruritus in individuals with NP who do not have a history of atopic dermatitis or other pruritic skin disorders. These causes may include chronic kidney disease, liver disease, thyroid disease, HIV infection, parasite infestation, or malignancy. The initial laboratory and imaging evaluation may consist of:

  • Complete blood cell count
  • Liver function tests
  • Blood urea nitrogen and creatinine
  • Thyroid-stimulating hormone
  • HIV test
  • Urinalysis
  • Stool examination for ova and parasites
  • Chest radiograph

 

Management:

A complex and multi-modal strategy is necessary for the treatment of NP which includes:

  • Instructing patients on various ways to reduce skin itching and scratching 
  • Treating pruritus symptoms 
  • Topical or systemic therapies that target the itch-scratch cycle and minimize skin lesions

Common treatment options include: 

  • Topical emollients
  • Topical steroid cream or ointment — usually ultrapotent steroid and applied under hydrocolloid dressing or paste bandage occlusion, or steroid impregnated tape
  • Topical capsaicin and tacrolimus 0.1% ointment for itch
  • Local corticosteroid injection into the nodules to reduce inflammation
  • Sedating oral antihistamines
  • Phototherapy with either UVB or photochemotherapy (PUVA)
  • Systemic treatment with gabapentin, pregabalin, and naltrexone
  • Occasionally, antibiotics may be used to treat locally infected skin (cellulitis).

 

Written by:

Mashael Alanazi, Medical student.

Revised by:

Maee Barakeh, Medical student.

References:

DermNet

Uptodate

Molluscum Contagiosum

It is a benign, often asymptomatic viral infection of the skin, with no systemic manifestation. It is caused by molluscipoxvirus, a part of the poxviridae family which is a family of double-stranded DNA viruses. It is most common in childhood and early adolescence. 

 

Risk Factors:

As mentioned, the etiology is due to molluscipoxvirus, but we have several risk factors associated with this disease. These risk factors include immunosuppression state, active atopic dermatitis, hot and humid climates, and crowded living conditions. Generally, those with poor immune function and immunocompromised skin are at risk for molluscum contagiosum.

 

Mode of Transmission:

Molluscum contagiosum can be transmitted due to direct contact during sports or other activities including sexual contact, indirect skin contact, or with fomites which are any object that can transmit the organism. Lastly, auto-inoculation can transmit the lesion by scratching or any trauma resulting in linear lesions (Pseudo koebner phenomenon).  

 

Clinical Features:

The incubation period will usually be 2-6 weeks or more. Usually, the patient will come complaining of single or multiple non-tenders, skin-colored, pearly, dome-shaped papules with central umbilication. The lesion will be 2–5 mm in diameter and contain a caseous plug which if you squeeze will produce white cheesy fluid that consists of molluscum bodies. 

The usual lesions in children will be the face, trunk, and limbs, in contrast to adults where the lesions will be usually in the abdomen, inner thighs, and genitalia. Finally, patients with HIV will usually have widespread and large (more than 15 mm) lesions. 

 

Diagnosis:

Molluscum contagiosum is usually recognized by its characteristic clinical appearance (dome-shaped papules, often with central umbilication), and is distinctive on dermatoscopy which will show white molluscum bodies can often be expressed from the center of the papules. Sometimes, the diagnosis is made on skin biopsy. Histopathology will show the following features, acanthosis, cup-shaped invagination, and molluscum bodies (Henderson-Paterson bodies) which are keratinocytes with eosinophilic intracytoplasmic inclusion bodies containing viral particles. 

 

Management:

Spontaneous remission is expected, however active treatment is considered in sexually transmitted molluscum, immunocompromised individuals or upon parental request.  

The first line of treatments includes cryotherapy and curettage which has an immediate resolution of the lesion. Both treatments  are well tolerated by adults.

Cantharidin which is a blistering agent can be applied directly to lesions, and a small blister will form which will result in the disappearance of the lesion and healing without scarring. Podophyllotoxin can also be used directly on the skin. 

Immunocompromised patients or individuals with refractory disease will usually benefit from Cidofovir, Interferon-α, and Imiquimod. Other treatments for molluscum include potassium hydroxide, salicylic acid, topical retinoids, oral cimetidine, and pulsed dye lasers.

 

Complications: 

It includes secondary bacterial infection from scratching, conjunctivitis, disseminated secondary eczema, and scarring.

 

Prevention: 

Patients should be instructed to do the following, keep good hygiene, cover the lesion, do not share towels, clothing, or other personal items, and avoid sexual activity until the resolution of the lesion.

 

 

Written by:

Mohammed Alahmadi, Medical Student. 

Revised by:

Maee Barakeh, Medical Student. 

References:

DermNet

Dermatology Essentials by Bolognia

Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology

UpToDate  

Keratoacanthoma

Keratoacanthoma (KA) is a commonly encountered skin tumor that presents a captivating clinical challenge due to its rapid growth and locally destructive nature. While some cases of KA may regress spontaneously, they often leave behind visible scarring. However, the clinical presentation of KA can closely resemble well-differentiated squamous cell carcinoma (SCC), leading to unpredictability in its course.

Pathophysiology:

The precise pathophysiological mechanisms underlying keratoacanthoma are not fully understood. However, it is widely believed to arise from the hair follicles or the sebaceous glands. Several hypotheses have been proposed, including the role of ultraviolet radiation, genetic factors, viral infections, and immune dysregulation in the development of keratoacanthoma.

Clinical Features:

Keratoacanthoma typically presents as a rapidly growing dome-shaped nodule with a central crater filled with keratin debris. It commonly occurs on sun-exposed areas of the body, such as the face, scalp, ears, and dorsum of the hands. Initially, the lesion may resemble a pimple or a wart, but it rapidly enlarges over a period of weeks to months. The growth phase is followed by a stabilization phase, during which the lesion may persist for several months before spontaneously regressing.

Diagnosis:

The diagnosis of keratoacanthoma is primarily based on clinical examination and histopathological evaluation. A thorough physical examination, including a dermatoscopic assessment, can aid in distinguishing keratoacanthoma from other skin lesions. Dermoscopy often reveals a central plug, keratin-filled crater, and peripheral keratotic rim. To confirm the diagnosis, a skin biopsy is performed, and the specimen is sent for histopathological analysis. Microscopically, keratoacanthoma is characterized by a well-demarcated cup-shaped lesion with a central keratin-filled crater, hyperkeratosis, and proliferation of keratinocytes.

Management:

The management of keratoacanthoma depends on various factors, including the size, location, and individual patient characteristics. Several treatment options are available, and the choice of therapy should be tailored to the specific case. Here are some commonly employed management strategies:

1. Observation: In some cases, keratoacanthomas may undergo spontaneous regression without any intervention. Close observation with regular follow-up visits may be appropriate for small, asymptomatic lesions in low-risk areas.

2. Surgical Excision: Surgical excision is a commonly employed treatment modality for keratoacanthoma. It involves complete removal of the lesion with a margin of healthy tissue to reduce the risk of recurrence. This approach is particularly suitable for larger or symptomatic tumors.

3. Mohs Micrographic Surgery: Mohs surgery is a specialized technique that ensures the highest cure rate while preserving as much healthy tissue as possible. It is often recommended for lesions in cosmetically sensitive areas or those with aggressive features.

4. Topical Therapies: In select cases, topical treatments such as imiquimod cream or 5-fluorouracil may be employed. These medications stimulate the immune system or inhibit cell proliferation, leading to tumor regression.

5. Cryotherapy: Cryotherapy involves freezing the lesion with liquid nitrogen. It is commonly utilized for smaller keratoacanthomas and can be performed in an outpatient setting.

Written by:

Deemah AlHuraish, medical student.

Revised by:

Maee Barakeh, medical student.

References:
DermNet

NCBI

AOCD