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Chapter 59. Mucha-Habermann Disease

Definition

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, the 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.

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Course

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 pink 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 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 progress very slowly into redder papules that become scaly. In time they progress to scaly brown papules that, after 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 thereafter. 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 in terms of 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 keratocytes are present throughout an epidermis that displays ballooning and spongiosis. Parakeratosis that may house neutrophils is 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.

Pityriasis lichenoides chronica 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 epidermis, just a hint, if that, of ballooning and spongiosis, and subtle mounds of parakeratosis. The lesions of this scaly clinical presentation never progress to 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.

Despite some similarities in the clinical appearance between Mucha-Habermann disease and lymphomatoid papulosis, the two are unrelated. Mucha-Habermann disease is an inflammatory process, whereas lymphomatoid papulosis is a lymphoma.

Therapy

Click here for an up-to-date discussion of therapeutic management presented in Therapeutic Strategies in Dermatology.

Distribution and Individual Lesions

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Fig. 59-1

Widespread purpuric papules.


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Fig. 59-2

Widespread purpuric papules.


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Fig. 59-3

Purpuric papules, some covered by hemorrhagic crusts.


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Fig. 59-4

Discrete papules.


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Fig. 59-5

Discrete purpuric papules, one of them covered by a hemorrhagic crust.


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Fig. 59-6

Purpuric papules.


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Fig. 59-7

Smooth-surfaced erythematous papules, scaly papules, and hemorrhagic crusted papules.


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Fig. 59-8

Erythematous papules, purpuric papules, scaly and crusted papules, and scale-crusts.


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Fig. 59-9

Smooth-surfaced, scaly, and crusted papules.


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Fig. 59-10

Scaly papule.


Variations

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Fig. 59-11 A

Innumerable widespread purpuric papules, many ulcerated and covered by hemorrhagic crusts, of pityriasis lichenoides acuta.


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Fig. 59-11 B

Innumerable widespread purpuric papules, many ulcerated and covered by hemorrhagic crusts, of pityriasis lichenoides acuta.


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Fig. 59-12

Hemorrhagic macules, patches, and papules, many ulcerated and covered by hemorrhagic crusts (“fulminant”).


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Fig. 59-13

Smooth-surfaced papules and an ulcerated papule covered by an eschar of pityriasis lichenoides acuta.


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Fig. 59-14

Ulcers, eschars, and scars of pityriasis lichenoides acuta.


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Fig. 59-15

Discrete papules of pityriasis lichenoides chronica.


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Fig. 59-16 A

Many papules covered by chalk-white scales of pityriasis lichenoides chronica.


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Fig. 59-16 B

Many papules covered by chalk-white scales of pityriasis lichenoides chronica.


New! Additional Images

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Fig. 59-17 A

Mucha-Habermann disease: Macules and papules, some of them purpuric and others covered by hemorrhagic crust. Note how oval are most of the lesions and how different the condition actually is from lymphomatoid papulosis.


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Fig. 59-17 B

Mucha-Habermann disease: Macules and papules, some of them purpuric and others covered by hemorrhagic crust. Note how oval are most of the lesions and how different the condition actually is from lymphomatoid papulosis.