| Mucha-Habermann's Info! |
This first section is an abridged version. There are two full entries below.
Mucha-Habermann's disease causes sores on the skin that are like little ulcers somewhat like chicken pox. They can get larger and have black scabbed tops. The name, varioliformis, indicates they can appear in varied forms. There is no known cause or cure for it. It is said to be non-contagious. The first symptoms are somewhat like the flu with fever, fatigue and swollen glands. According to the dermatology texts there are few systemic symptoms, however this is one of the areas of interest in research. Also according to the texts, it is most common in men and usually occurs before 40 years of age though it has been seen infrequently in all age groups and both sexes. Treatments that have been tried include all forms of hydrocortizone skin preparations, light therapy, antibiotics and antihistamines. The lives of the people with this disease can be affected because of the appearance of the lesions. Dress, areas where they can work, other people's response to them, etc. may be affected. All of these factors make this disease appear in the research list of BEG, etc. at this time.
(Note: Do you have another disease that should be studied? If so, leave a note on the question page.)
From: Dermatology in General Medicine, Third Edition by Fitzpatrick, et al. (references and footnotes not included) [Comments or definitions are mine]
Definition
Pityriasis lichinoides is a distinctive cutaneous eruption characterized by acute and/or chronic morphological phases. The episodic form was described by Mucha and named pityriasis lichenoides et varioliformis acuta (PLEVA) by Habermann; It is also known as acute parapsoriasis and Mucha-Habermann's disease. The chronic form was originally described as a type of chronic parapsoriasis by Brocq. It is also called pityriasis lichenoides chronica and guttate psoriasis.
Etiology
The cause is unknown. Clustered, sporadic outbreaks of pityriasis lichenoides have suggested an infectious etiology; however, the successful isolation of bacterial, parasitic, or viral agents, and human-to-human transmission have not yet been reported. Although the ultrastructural observation of the tubular aggregates in the epidermal cells has been made, suggesting the presence of viral particles, this finding may represent a nonspecific cytoplasmic reaction to injury.
Both the clinical resemblance to certain forms of cutaneous necrotizing vasculitis and the presence of increased numbers of histiocytes and lymphocytes about blood vessels have suggested an immunologic pathogenisis. The infiltrating cells in both the epidermis and dermis are T lymphocytes with the cytotoxic/suppressor phenotype being more numerous that the helper/inducer phenotype. Moreover, a decrease in epidermal Langerhans cells was noted. Although circulating immune complexes have been described in this disease, there is yet insufficient evidence to correltate them with the immuniglobulins and complement found in the affected vessels by direct immunoflorescence.
Histopathology omitted.
Clinical Manifestations: In the acute form, pityriasis lichenoides is a polymorphous eruption[this means many forms] characterized by the abrupt onset of skin lesions that become generalized. The typical lesion is a papule which can vary in size from 2mm to over 1 cm. They are round or oval, firm, and vary in color from waxy pink to red-brown. Some are covered with a mica-like scale, whereas others contain minute vesicles at their summits. The papules usually show foci of hemorrhage and occasionally become necrotic and ulcerative. The lesions resolve with transient hyperpigmentation, hypopigmentation, and/or varioliformis [like chickenpox] scars.
The eruption appears in successive crops. At any one time all stages of development and regression may be present in the same patient. The sites of predilection are the anterior aspect of the trunk and the flexor aspects of the extremities. Rare instances of oral and penile mucous membrane lesions have been reported.. The eruption usually begins during the first three decades of life but occasional reports extend the range from infancy to old age. The disease is more common in males. Mild constitutional symptoms of fever, malaise, and lymphadenopathy may be present. The eruption is usually asymptomatic but pruritis with burning may occur. In rare instances, PLEVA has been associated with a necrotizing vasculitis characterized by fever and sudden appearance of cutaneous ulcers.
In the acute form of PLEVA, resolution may occur within three months, but more often the acute episodes occur for indefinate periods and occasionally the eruption changes to that seen in the chronic form. Despite the prolonged course, neither serious sequelae nor systemic involvment has been reported. [This is one of the areas I feel more information is needed.]
Treatment: There is no single treatment for pityriasis lichenoides. The administration of H1 antihistamine preparations may alleviate lesional pruritus. In occasional patients with the severe acute form, the administration of systemic steroids or methotrexate has been reported to be effective. The administration of systemic steroids is not recommended in the chronic form. The administration of tetracycline and erythromycin has been reported to be beneficial, but the variabile course of this disease makes the assessment of its effectiveness difficult. Exposure to sunlight or phototherapy (UVB) or photochemotherapy (PUVA) has been noted to be beneficial to patients with the acute form. The chronic form may respond to erythemogenic doses of UVB or PUVA.
These are some abstracts about PLEVA:
Title
Pityriasis lichenoides in children: clinicopathologic review of 22 patients.
Author
Romaní J; Puig L; Fernández Figueras MT; de Moragas JM
Address
Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Source
Pediatr Dermatol, 1998 Jan, 15:1, 1-6
Abstract
Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin, with an autoinvolutive course, that can occur in pediatric patients. Traditionally, acute and chronic variants have been described, but other special forms of presentation have been reported. We reviewed the clinical records and histopathologic specimens of all pediatric patients diagnosed with PL in our hospital from 1980 to 1995 to assess the clinicopathologic features of this disorder in our environment. Twenty-two of the 118 cases reviewed were pediatric patients less than 15 years old (12 males and 10 females, 18.6% of all patients). Their ages ranged from 3 to 15 years, with a mean of 9.3 years. Most of the patients (72%) had the chronic variant of the disease, while the remainder had an acute course. One patient suffered from acute ulceronecrotic PL. Systemic treatments prescribed were erythromycin in eight patients, PUVA in five patients, and methotrexate in one patient. Three patients had a prolonged course with more than two episodes. Acute and chronic PL are polar extremes, but individual cases cannot be classified only on the basis of histopathologic data, since coexistence of lesions in different stages of evolution can lead to sampling bias. Acute ulceronecrotic forms and the presence of a variable degree of cellular atypia in the infiltrate are liable to cause differential diagnostic problems with lymphomatoid papulosis (LP), which cannot be completely resolved on the basis of T-cell receptor clonal rearrangement detection.
Language of Publication
English
Title
Clinical and histologic features of pityriasis lichenoides et varioliformis acuta in children.
Author
Longley J; Demar L; Feinstein RP; Miller RL; Silvers DN
Address
Department of Pathology, College of Physicians and Surgeons of Columbia University, New York.
Source
Arch Dermatol, 1987 Oct, 123:10, 1335-9
Abstract
Pityriasis lichenoides et varioliformis acuta (PLEVA) is commonly thought of as a disease of young adults, yet we identified five cases, involving patients who were 3, 5, 6, 8, and 11 years of age, among 13,000 consecutive specimens submitted to a general dermatopathology laboratory during a 15-week period. The clinical and histologic features of PLEVA in our cases were similar to those reported for adults, except that no lesions were observed on the scalp or mucous membranes of children. A high index of suspicion and biopsy specimens of suspected lesions are often needed to differentiate PLEVA from other papular and crusted eruptions seen in the pediatric age group. These include reactions to arthropods, Gianotti-Crosti syndrome, varicella, and erythema multiforme. Histologically, papular eczema and pityriasis rosea may be misdiagnosed as PLEVA.
Language of Publication
English
Title
Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation.
Author
Hood AF; Mark EJ
Source
Arch Dermatol, 1982 Jul, 118:7, 478-82
Abstract
To assess the specificity of the histopathologic features in the diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA), we reviewed the clinical manifestations and courses of 42 patients for whom this diagnosis was suggested in the pathology report. The histologic diagnosis of PLEVA was clinically substantiated in 16 of these 42 cases. Of the 26 cases in which PLEVA was erroneously diagnosed histologically, the correct clinical diagnosis was suggested before biopsies were done in 21 instances. In the five remaining cases, both the prebiopsy clinical diagnosis and the pathologic diagnosis proved to be incorrect. Pityriasis rosea, insect bites, and eczematous dermatitis accounted for the majority of the cases that histologically mimicked PLEVA. The constellation of histologic findings described in PLEVA (presence of intraepidermal lymphocytes and erythrocytes, dermal hemorrhage, and so-called lymphocytic vasculitis) is not specific and may be seen in a variety of dermatologic disorders.
Language of Publication
English
Title
Papulosquamous diseases: a review.
Author
Fox BJ; Odom RB
Source
J Am Acad Dermatol, 1985 Apr, 12:4, 597-624
Abstract
Papulosquamous diseases are a heterogeneous group of disorders whose etiology primarily is unknown. The nosology of these disorders is based on a descriptive morphology of clinical lesions characterized by scaly papules and plaques. The major entities in this group include psoriasis, parapsoriasis (including pityriasis lichenoides et varioliformis acuta), lichen planus, lichen nitidus, lichen striatus, pityriasis rosea, pityriasis rubra pilaris, seborrheic dermatitis, and the Gianotti-Crosti syndrome. Many other conditions may become papulosquamous and should be considered in the differential diagnosis.
Language of Publication
English
Title
Human herpesvirus 7 in patients with pityriasis rosea. Electron microscopy investigations and polymerase chain reaction in mononuclear cells, plasma and skin.
Author
Drago F; Ranieri E; Malaguti F; Battifoglio ML; Losi E; Rebora A
Address
Institute of Dermatology, University of Genoa, Italy.
Source
Dermatology, 1997, 195:4, 374-8
(Comment from webmaster about article below: I have not found a direct relationship between Pityriasis Rosea and PLEVA except as a grouping of skin diseases with similar symptoms.)
Abstract
BACKGROUND: Clinical evidence suggests a viral etiology for pityriasis rosea (PR). OBJECTIVE: To evaluate human herpesvirus (HHV)-6 and HHV-7 as candidates for the etiology of PR.
METHODS: Blood and skin tissue from 12 patients with acute PR, and 12 patients with other dermatoses were studied, as well as blood samples from 25 healthy persons. Serum interferon (IFN)-alpha and IFN-gamma were analyzed by ELISA. Analysis of morphological changes in cocultured peripheral blood mononuclear cells (PBMC) and electron microscopy (EM) to identify viral particles were performed. Polymerase chain reaction (PCR) with specific primers for HHV-6 and HHV-7 DNA sequences was performed on the plasma and PBMC of patients and healthy controls and on the skin of patients with PR and other skin diseases. RESULTS: PR plasma contained detectable IFN-alpha and IFN-gamma, whereas plasma from controls did not. PBMC from PR patients showed ballooning cells and syncytia after 7 days in culture whereas PBMC from controls and recovered PR patients did not. This cytopathic effect was also documented in a PR patient who relapsed and in Sup-T1 cell cultures inoculated with the cell-free supernatant from centrifuged cultured PBMC; in this supernatant, herpesvirus, virions were detected by EM, PCR identified HHV-7 DNA in PBMC, plasma and skin from all patients with active PR and in the PBMC only of 5 patients tested 10-14 months later. Weaker signals of HHV-7 DNA were detected in PBMC of 11 controls, but not in their plasma. Skin was negative for HHV-7 in all control specimens.
CONCLUSIONS: Although the detection of HHV-7 DNA in PBMC and tissues does not prove directly a causal role, HHV-7 DNA in cell-free plasma corresponds to active replication which supports a causal relationship. We propose that PR is a clinical presentation of HHV-7 reactivation.
Language of Publication
English
Some other links that contain pictures and information and may be of interest:
This site contains information about one person with PLEVA http://www.myskin.4t.com/mystory.htm and has good pictures of the problem here http://www.myskin.4t.com/myskin.htm I have not copied it and hope it stays available.
http://www.skinsite.com/info_pityriasis_lichenoides.htm
http://www.dermnet.org.nz Press search on the left index, insert PLEVA in the search box, press enter and two document listings will come up. Pick one or both to view. Both articles have pictures.
http://tray.dermatology.uiowa.edu/DermImag.htm http://tray.dermatology.uiowa.edu/PLEVA001.htm http://tray.dermatology.uiowa.edu/PLEVA002.htm http://tray.dermatology.uiowa.edu/PLEVA003.htm http://tray.dermatology.uiowa.edu/PLEVA004.htm http://www.derma.med.uni-erlangen.de/bilddb/diagnose/englisch/i696240.htm http://www.meddean.luc.edu/lumen/MedEd/medicine/dermatology/melton/pity2.htm http://www.medic.mie-u.ac.jp/derma/bilddb/diagnose/i696250.htm
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