Case of the Month
30 year old man present with a 2 week history of pruritic papules and vesicles with purple crusts on his arms and trunk, and a low grade fever.
What's Your Diagnosis?
Diagnosis: Mucha–Habermann disease (a.k.a Pityriasis Lichenoides Varioliformis Acuta, commonly called “PLEVA”)
An inflammatory process in which lesions may erupt (pityriasis lichenoides et varioliformis acuta) and evolve rapidly from erythematous macules and papules to become purpuric papules papulovesicles that may be hemorrhagic ulcers, eschars, and, eventually scars. Alternatively, macules may evolve more slowly (pityriasis lichenoides chronica) to become scaly papules. In some patients, rapidly developing and slowly evolving lesions may be present concurrently, e.g., purpuric papules and scaly papules may coexist.
The condition commonly affects children and young adults.
Lesions of Mucha – Habermann disease evolve in two very different ways, namely, as an efflorescence and at a petty pace. The former (acuta) begins as macules that quickly become reddish papules. They tend to transform rapidly into papulovesicles, which become necrotic and ulcerate to form eschars that heal with scars. Sometimes the process is so fulminant that hemorrhagic vesicles, and even hemorrhagic bullae, monopolize.
The expression of Mucha – Habermann disease is characterized by rapid development of lesions that often culminate in vesicles is known formally as "pityriasis lichenoides et varioliformis acuta." An exaggeration of the process results in "fulminant" Mucha-Habermann disease with widespread hemorrhagic blisters that ulcerate deeply and become covered by an eschar. Patients with this severely necrotizing form of the disease may die of it.
In contrast, slowly evolving lesions of Mucha-Habermann disease (chronica) begin as pink macules that process very slowly to red papules that become scaly. In time they progress to scaly brown papules that, many months, become hyperpigmented macules. The slowly evolving expression of Mucha-Habermann disease is known as "pityriasis lichenoides chronica."
In short, rapidly-evolving lesions of Mucha-Habermann disease can become vesicles within days, eschars within a few weeks, and scars after that. Slowly evolving lesions of Mucha-Habermann disease play out their lives as scaly papules over many months and sometimes years. The disease itself is unpredictable regarding longevity; its acute expression is sometimes finished in weeks, whereas its chronic manifestation sometimes sputters for years.
Integration: Unifying Concept
Clinically and histopathologically, Mucha-Habermann disease is a single distinctive pathologic process. It is not unique in being associated with lesions that may be accelerated to become blisters or decelerated to remain scaly papules. The same is true of lichen planus, which may become vesicular (bullous lichen planus) or be scaly papules, of psoriasis, which may become pustular (pustular psoriasis) or be scaly papules or plaques, and of lupus erythematosus (lupus dermatitis), which may be bullous (bullous lupus erythematosus) or simply scaly papules and plaques. Papules of pityriasis lichenoides acuta are characterized histopathologically by a wedge-shaped, superficial and deep, perivascular infiltrate of lymphocytes, and by lymphocytes scattered along the dermoepidermal junction in association with vacuolar alteration. Individual necrotic keratinocytes are present throughout an epidermis that displays ballooning and spongiosis.Parakeratosis that may house neutrophils also present. If extravasated erythrocytes are numerous, the lesions will be purpuric. If ballooning and spongiosis eventuate in vesiculation, a papulovesicle is the result. When epidermal necrosis is extensive, and ulceration supervenes, an eschar comes into being. If ulceration is deep enough, a scar results.
Parakeratosis lichenoides acuta is typified histopathologically by a superficial perivascular infiltrate of lymphocytes, lymphocytes in association with vacuolar alteration along the dermoepidermal junction, only a few necrotic keratinocytes within the the epidermis, just a hint, if that, of ballooning and spongiosis, and subtle mounds of parakeratosis. The lesions of this scaly clinical presentation never progress into vesiculation, ulceration, eschars, or scars.
Not surprisingly, some patients with pityriasis lichenoides show acute and chronic lesions concurrently. The fulminant expression of the process is simply a caricature of pityriasis lichenoides acuta, but can be severe and Life-threatening. The fulminant expression can be associated with high fever, GI, CNS,Pulmonary, and cardiac involvement/complications.
Despite some similarities in the clinical appearance of Mucha-Habermann disease and lymphomatoid papulosis, the two are unrelated. Mucha-Habermann disease is an inflammatory process, whereas lymphomatoid papulosis is a lymphoma.
Oral erythromycin, tetracycline, as well as phototherapy (nbUVB), are indicated for the acute form, and methotrexate for fulminant form. Other treatments that have been reported to be effective include, but are not limited to: topical Tacrolimus, Dapsone, Infliximab, and IVIG.
Here is another picture of a child with PLEVA
A CLINICAL ATLAS OF 101 COMMON SKIN DISEASES
With Histopathologic Correlation
A. BERNARD ACKERMAN, HELMET KERL, JORGE SANCHEZ, YING GUO, ANGELIKA HOFER, PAUL KELLY, TETSU KIMURA, GIOVANNI BORRONI,CHARLES CRUTCHFIELD, VOLKER STEINKRAUS, WOLFGANG WEYERS
For more dermatology cases-of-the-month visit Dr. Crutchfield's Case of the Month Archive
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