What is transient acantholytic dermatosis?

Transient acantholytic dermatosis, also known as Grover disease, is a frequent, acquired, itchy, truncal rash that histopathologically shows acantholysis. It is more common in Caucasian men over 50 with sun damaged skin. It is less prevalent in women and young adults.
Sun exposure, sweating, fever, cancer, and being in the hospital or bedridden are all risk factors for transient acantholytic dermatosis.

It is becoming more common with the use of BRAF inhibitors such as vemurafenib and dabrafenib monotherapy, as well as cytotoxic chemotherapeutic agents.

 

Causes :

The cause of transitory acantholytic dermatosis remains unknown. Because transitory acantholytic dermatosis is frequently associated with skin occlusion, heat, and sweating, one theory suggests that it is caused by sweat duct injury and occlusion. Drug-induced transitory acantholytic dermatosis may be due to medication or its metabolites being secreted in sweat, which has toxic effects on the epidermis, resulting in acantholysis and dyskeratosis. BRAF-induced transitory acantholytic dermatosis may be caused by keratinocyte proliferation triggered by MAP-kinase activation. SARS-CoV-2 was detected on immunohistochemistry in the sweat gland epithelium and cutaneous vasculature of a COVID-19 patient.

Clinical features :

Transitory acantholytic dermatosis frequently begins abruptly. Some (but not all) research indicates that it is more common in the winter than in the summer . The most frequently affected areas are the central back, mid-chest, and upper arms. Lesions consist of tiny red, crusty, or eroded papules and vesicles. The rash is extremely pruritic.

Diagnosis :

Transient acantholytic dermatosis is typically diagnosed clinically; however, a skin biopsy may be required. Transient acantholytic dermatosis has a distinct pathology that includes acantholysis (separated skin cells) and/or dyskeratosis (abnormally rounded skin cells). Spongiotic dermatitis might also be noticed.

 

Treatment :

Transient acantholytic dermatosis has no cure; however, the following tips may help relieve the itch and speed up the healing process:

• Keep cool, as sweating may cause more itchy areas.
• Menthol- and camphor-based moisturizing creams or antipruritic lotions can help lessen the desire to scratch.
• Cryotherapy
• Topical trichloroacetic acid
• Topical steroid
• Topical steroid/vitamin D analogue combination ointment
• Oral retinoids
• Phototherapy and photochemotherapy

 

Complications :

Dermatitis can complicate transient acantholytic dermatosis, which commonly appears in a discoid pattern with round or oval, dry, or crusted plaques. The plaques begin on the chest and back and may migrate to the limbs.

Prognosis :

Transient acantholytic dermatosis has a variable duration, but it usually resolves spontaneously within 2-4 weeks. It can come and go, with seasonal variations. Transient acantholytic dermatosis, despite being named transient, is frequently recurring and can be chronic, lasting for years.

 

Written by:

Atheer Alhuthaili , medical student.

Revised by:

Maee barakeh , medical student.

References:

DermNet.

Medscape.

Definition:

Erythema nodosum is a form of panniculitis, which is an inflammatory condition that specifically targets the subcutaneous fat. It appears on the anterior shins as painful red nodules. Occasionally, they affect the thighs and forearms.

 

Etiology:

Erythema nodosum is a type of hypersensitivity reaction that occurs in approximately 55% of patients, and its etiology is currently unknown. In some situations, there is an association to an infection, medication, inflammatory disease, or cancer.

• Pharyngitis: caused by streptococcal bacteria or viral pathogen.
• Yersinia infection: can lead to symptoms such as diarrhea and stomach pain.
• Chlamydia infection.
• Fungal infections: histoplasmosis and coccidioidomycosis.
• Parasitic infection: amoebiasis and giardiasis.
• Viral: such as herpes simplex, viral hepatitis and human immunodeficiency virus (HIV) infection.

 

Clinical features:

Erythema nodosum is characterized by the presence of painful, red, and swollen nodules that appear under the skin. These nodules can range in size from 3 to 20 cm and typically develop over a period of one to several weeks. The symptoms include fever and joint pain. Swelling and pain in the ankle occur in 50% of cases and might last for many weeks. It may also affect other joints, such as the knees.

The nodules are primarily located on the anterior lower legs, knees and arms.
Their shape can be described as ill-defined, warm, oval, round, or arciform, and they do not exhibit ulceration.
The nodules exhibit an initial color ranging from bright to deep red.
Erythema contusiformis is a term used to describe the development of violaceous, brownish, or yellowish bruise-like marks that dissolve on their own within eight weeks.
Erythema nodosum does not result in permanent scarring.

 

Diagnosis:

Erythema nodosum is primarily diagnosed through clinical examination and confirmed by laboratory tests and histology. Erythema nodosum pathology primarily manifests as inflammation of the septa between subcutaneous fat lobules in the absence of vasculitis.

 

Differential diagnosis:

Panniculitis can manifest as either mainly septal, characterized by inflammation between lobules, or lobular, characterized by inflammatory cells within subcutaneous fat lobules. Septal and lobular inflammation can coexist.

The nodules caused by mostly septal panniculitis include:

-Different types of scleroderma
-Medium vessel vasculitis, such as that caused by polyarteritis nodosa, is characterized by the presence of sensitive subcutaneous nodules accompanied by ulceration, necrosis, livedo racemosa, fever, joint discomfort, myalgia, and peripheral neuropathy.
-Necrobiosis lipoidica
-Eosinophilic panniculitis
-Rheumatoid nodule.

The nodules caused by mostly lobular panniculitis include:

-Connective tissue illness, such as panniculitis linked with cutaneous lupus erythematosus.
-Erythema nodosum leprosum.
-Pancreatic panniculitis is a condition characterized by the development of subcutaneous nodules that may become ulcerated or fluctuant. The laboratory tests reveal increased levels of lipase, amylase, and trypsin.
-Traumatic panniculitis
-Nodular vasculitis, also known as erythema induratum, is a condition characterized by the presence of ulcerated and draining nodules that specifically affect the posterior calves.
-Lipodermatosclerosis is a condition that occurs as a result of venous insufficiency.
-Subcutaneous fat infection caused by bacteria, mycobacteria, or fungus, resulting in the formation of ulcerated, fluctuant, and draining abscesses.

 

Treatment:

Treatment for erythema nodosum is determined by the primary cause of the condition. It is necessary to address any underlying infection.

Methods for pain management may involve prolonged periods of rest, administration of colchicine, use of NSAIDs, and application of venous compression therapy.
After infection, sepsis, and cancer have been ruled out, a prescription for systemic corticosteroids (1 mg/kg daily) may be prescribed until the erythema nodosum resolves.

 

 

Written by:

Mashael Alanazi, Medical Student.

Revised by:
Maee Barakeh, Medical Student.

Resources:
DermNet

Capillaritis also known as pigmented purpura is a benign skin disease characterized by reddish-brown patches that are caused by leaky capillaries. While it can afflict anyone, children are rarely affected by capillaritis. It usually affects healthy individuals, but multiple factors including medications, foods, viral infections, and exercise could trigger it.

Causes: 

Capillaritis is thought to be idiopathically caused by a mechanism that irritates the capillaries which are near the skin’s surface. The pathophysiology behind this disease is due to the passage of blood cells through the gaps between the capillary walls resulting in petechial hemorrhage which fades away depositing haemosiderin in the the dermis’ uppermost part.

Clinical features:

Red-brown purpuric macules “cayenne pepper spots” are the characteristic features of capillaritis. These may be distributed over the body or cluster together forming a red patch that gradually disappears over weeks to months.

Types and variants:

Progressive pigmented purpura (schamberg disease): The most common type of capillaritis, presented by cayenne pepper spots which appear suddenly. Most commonly on the lower leg, with irregular distribution and no associated symptoms.

Doucas-Kapetanakis eczematid-like purpura (itching purpura): Similar to schamberg disease bit with itching. It can affect the trunk and upper limbs.

Gougerot-Blum purpura (pigmented purpuric lichenoid dermatosis): Characterized by thick and itchy patches similar to eczema.

Majocchi purpura (purpura annularis telangiectodes): Characterized by annular patches that spread gradually and form concentric rings.

Lichen aureus: Overlies varicose vein and is characterized by a persistent single brown patch.

Exercise-induced capillaritis: common in prolonged exercise during the summer. The lesions fade with time. The appearance of the lesions is associated with a burning sensation.

Diagnosis:

The diagnosis is made clinically. It can be confirmed by skin biopsy and dermatoscopy. Histology may reveal red cell extravasation, hemosiderin, perivascular lymphocytic infiltrate, and lichenoid infiltrate, these findings could be found in one or more variants.  In immunocompromised patients always rule out cutaneous T-cell lymphoma.

Management:

Usually, no treatment is required, and unfortunately, no cure is known. However, avoiding possible risk factors including medications, food preservatives, and artificial coloring agents could result in improvements. Topical steroids can be used for symptomatic treatment. Ascorbic acid and rutoside can be used also. Phototherapy may be effective but with recurrence risk. Elastic compression socks can be used in exercise-induced capillaritis. Ascorbic acid and rutoside can be used also.

Outcome:

Capillaritis can go away in a few weeks, come back occasionally, or last for years.

 

Written by:

Mohammed Alahmadi, Medical Student.

Revised by:

Maee barakeh, Medical student.
References:

DermNet

Andrews’ Diseases of The Skin, Clinical Dermatology

Dermatology, Illustrated Study Guide and Comprehensive Board Review by Sima Jain

British Association of Dermatologists

Definition:

Erythema multiforme is an immune-mediated illness that usually resolves on its own. It affects the skin and mucous membranes, and is characterized by the presence of “target” lesions. Erythema multiforme major can be distinguished from multiforme minor by the presence of significant mucosal involvement. Episodes can manifest as either solitary, recurrent, or persistent.

Etiology:

1-Approximately 90% of cases are triggered by infection, with HSV type 1 being the predominant cause. Additional infectious triggers include:

• HSV type 2
• Cytomegalovirus
• Epstein-Barr virus
• Influenza virus
• Vulvovaginal candidiasis
• SARS-CoV-2
• Orf.

2-Medications that can potentially cause erythema multiforme include:

• Antibiotics (including erythromycin, nitrofurantoin, penicillins, sulfonamides, and tetracyclines)
• Anti-epileptics
• Non-steroidal anti-inflammatory drugs
• Vaccinations (most common cause in infants).

3-Additional disorders that are commonly linked to erythema multiforme, particularly in cases of chronic disease, include:

• Inflammatory bowel disease
• Hepatitis C
• Leukaemia
• Lymphoma
• Solid organ cancer malignancy

Clinical features:

Cutaneous features:

• Cutaneous lesions initially appear on the periphery and then move centrally.
• The distribution is typically symmetrical, with a tendency to favor extensor surfaces.
• May cause pain, itching, or swelling.
• Initial lesions manifest as circular, red papules that subsequently progress into target-shaped lesions.

Target lesions are characterized by three concentric rings of color variation:

-A dark, central region of epidermal necrosis

-Encircled by a less swollen area

-Surrounded by an erythematous periphery.

Atypical lesions can coexist alongside typical lesions. Atypical lesions exhibit elevated surfaces with indistinct boundaries.

In severe disease, up to hundreds of lesions in varying developmental stages may be present making it challenging to distinguish characteristic lesions.

Mucosal features:

• Lesions form as blisters, which then rupture to reveal superficial erosions covered by a white pseudo-membrane.
• Erythema multiforme primarily affects the oral membranes, but can also manifest with urogenital and, infrequently, ocular lesions.
• When the mucous membrane is affected, it can cause pain and greatly restrict the ability to eat; these lesions may occur before or after skin lesions. 

 

The symptoms are anticipated to resolve spontaneously after 4 weeks from the start of the condition (or up to 6 weeks in cases of severe disease).

Diagnosis:

The diagnosis of acute erythema migrans (EM) is often established by evaluating the patient’s history and thorough physical examination of their clinical symptoms. Skin biopsies are helpful in confirming the diagnosis when there is uncertainty. Direct immunofluorescence studies may be necessary to rule out the presence of autoimmune blistering disease.

Differential diagnosis:

• Urticaria
• Viral exanthem
• Stevens-Johnson syndrome
• Fixed drug eruption
• Bullous pemphigoid
• Paraneoplastic pemphigus
• Polymorphous light eruption
• Rowell syndrome (erythema multiforme-like lesions in systemic lupus erythematosus)

Management:

Treatment is frequently unnecessary as episodes are generally self-limiting without any persistent complications. However, the presence of ocular involvement should always result in a referral to an ophthalmologist due to the potential for more severe consequences.

Management of symptomatic mild cases:

• Itch: To relieve itch or discomfort caused by skin lesions, oral antihistamines and/or topical steroids might be used.
• Pain: For mild mucosal involvement, oral washes containing antiseptic or local anesthetic might be used to relieve pain.

Additional therapies are dependent on the underlying cause:

• Infection: treat precipitating infections appropriately (notice that treating HSV does not change the course of a single episode of erythema multiforme significantly). 
• Offending medication: stop any offending medication and avoid in future.

Severe mucosal disease:

• Admission to the hospital might be necessary to support oral intake.

Chronic illness:

• A minimum duration of 6 months of continuous oral antiviral treatment (usually acyclovir/aciclovir), regardless of whether a definite cause has been determined.
• Achieving remission might be challenging and may necessitate trying a longer course of treatment or a different antiviral medication.

 

 

Written by:

Mashael Alanazi, Medical Student.

Revised by:

Maee Barakeh, Medical Student.

Resources:

DermNet

UpToDate

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