Febrile Ulceronecrotic Mucha-Habermann Disease Associated With Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature.

ABSTRACT: Mucha-Habermann disease (MHD) is an inflammatory skin disease characterized by polymorphous eruptions of erythematous, necrotic macules that have been reported for similarities to cutaneous T-cell lymphoma. Febrile ulceronecrotic MHD (FUMHD) represents a severe variant of MHD, marked by ulcers, hemorrhagic bullae, and systemic symptoms. Herein, we report a case of a severely atypical lymphomatoid expression of FUMHD associated with hemophagocytic lymphohistiocytosis (HLH). A previously healthy 21-year-old woman was admitted to the hospital with a rapidly progressive necrotic papular rash. Physical examination revealed right orbital swelling, bilateral hemorrhagic auricular bullae, and multiple ulcerative purpuric papulonodules on the trunk, face, and extremities. Biopsy indicated a dermal and subcutaneous infiltrate of atypical CD8 + lymphocytes with loss of CD5 and reduction in CD7 expression, along with features of lymphomatoid vasculitis. A diagnosis of a severely atypical lymphomatoid expression of FUMHD was made. The patient also met 7 of 9 HLH-2004 criteria, leading to a diagnosis of HLH. Positron emission tomography/computed tomography, flow cytometry, and rheumatologic workup were unremarkable. Treatment with an eight-week course of etoposide and dexamethasone for HLH led to rapid clinical improvement. Over time, her skin lesions regressed and eventually scabbed over to leave hyperpigmented scars, confirming the diagnosis of MHD. She has remained stable, off therapy for 4 years. Although potentially fatal, FUMHD often exhibits favorable outcomes and may resolve without recurrence, as in our patient. FUMHD should be considered in the differential diagnosis for patients presenting with cutaneous CD8 + necrotizing angiocentric lymphoproliferative disease complicated by HLH.

Mucha-Habermann disease: a pediatric case report and proposal of a risk score.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare inflammatory dermatological disease. A case of a 13-year-old boy with FUMHD possibly triggered by mycoplasma infection is presented. Based on FUMHD cases identified in a MEDLINE literature search, demographic, treatment, and outcome data were analyzed. An FUMHD mortality risk score is proposed based on the likelihood ratios of risk factors for a fatal outcome. Our FUMHD case had marked leukopenia and thrombocytopenia at admission. He recovered without systemic immunosuppressive treatment. Literature review revealed 119 FUMHD cases. Overall lethality was 14/119 (12%, CI 6-17%), and lethality in children was lower (1/54, 2%, CI 0-6%) compared to adults (13/65, 20%, CI 11-31%). Risk factors for a fatal outcome (likelihood ratio; P) were sepsis (24.97, P < 0.001), adult vs. pediatric patient age (11.19; P = 0.001), systemic involvement (19.97, P < 0.001), and mucosal involvement (4.58; P = 0.032). The proposed FUMHD mortality risk score = Age/10 + 4 + 4 (if systemic involvement) + 1 (if mucosal involvement) was discriminative (sensitivity 93%, specificity 77%). In FUMHD, immune-suppressive treatment intensity should be balanced against the mortality risk, as infectious complications are a frequent cause of death.

Rhabdomyomatous mesenchymal hamartoma presenting as telangiectasia in a 57-year-old man.

Rhabdomyomatous mesenchymal hamartoma (RMH) is a rare congenital malformation of the dermis and subcutaneous tissue. Usually, RMH occurs in the midline of the face and neck region. We described a case of RMH presenting as telangiectasia in a 57-year-old man with a history of pityriasis lichenoides chronicus. Histopathological examination revealed a subepidermal haphazard proliferation of striated muscular tissue perpendicular to the epidermis. These bundles of striated muscular tissue were admixed with adnexal structures. The diagnosis was consistent with RMH. RMH is more common in the neonatal period or in young children, but we should consider it as part of a differential diagnosis in older adults as well.

Mycosis Fungoides Preceding Pityriasis Lichenoides et Varioliformis Acuta by Twelve Years in a Pediatric Patient.

ABSTRACT: A 15-year-old boy presented to the pediatric dermatology department with long-standing patch stage CD8+ mycosis fungoides and subsequent development of recurrent pityriasis lichenoides et varioliformis acuta eruptions. There have been rare reports of patients with chronic, recalcitrant pityriasis lichenoides developing mycosis fungoides, but we believe this to be the second case of mycosis fungoides preceding a diagnosis of pityriasis lichenoides, and the first case reported in the pediatric population.

Pityriasis Lichenoides Chronica in a Patient With Ankylosing Spondylitis Treated With Etanercept.

Pityriasis lichenoides is a scarce cutaneous disorder with unknown etiology. It contains a range of clinical manifestations including acute papular lesions that quickly grow into pseudo vesicles and central necrosis to small, scaling, benign-appearing papules.1,2.

A case of febrile ulceronecrotic Mucha-Habermann disease with comorbidities.

Febrile ulceronecrotic Mucha-Habermann disease is a very rare and severe variant of pityriasis lichenoides et varioliformis acuta. Adult cases are difficult to diagnose as in the early course they can mimic erythema multiforme or lymphomatoid papulosis. We report a case of a 38-year-old woman who presented with 90% body surface area involvement, fever, diarrhea, malaise and associated comorbidities. She was treated with systemic steroids and methotrexate but suffered a fatal outcome. So far, a total of 65 cases are reported in the literature.

Frequent relapses in a child with nephrotic syndrome due to PLEVA.

A seven-year-old boy with nephrotic syndrome presented with a frequent rash along with relapse of nephrotic syndrome. Clinical and histological features were suggestive of pityriasis lichenoides et varioliformis acuta (PLEVA). Treatment of the condition with doxycycline led to the cure of the lesions as well as the relapses.

[Polymorphic and itchy rash in a 21-year-old woman]. / Polymorphes und juckendes Exanthem bei einer 21-jährigen Frau.

Pityriasis lichenoides et varioliformis acuta (PLEVA) is a common inflammatory disease which usually occurs in children and young adults. Characteristic is a polymorph clinical picture with papules, erosions, ulcers and haemorrhagic crusts compatible with many differential diagnoses. We report about a patient with PLEVA and describe possible differential diagnoses.

Severe Mucha-Habermann-Like Ulceronecrotic Skin Disease in T-Cell Acute Lymphoblastic Leukemia Responsive to Basiliximab and Stem Cell Transplant.

A 5-year-old girl with T-cell acute lymphoblastic leukemia (T-ALL) developed a progressive eruption of crusted papules and ulcerative plaques involving 80% of her body surface area with histopathology consistent with febrile ulceronecrotic Mucha-Habermann disease (FUMHD), although multiple specimens also contained clonal leukemic cells. Her skin disease was refractory to many classic treatments for FUMHD, including methotrexate, and became so severe that concern about superinfection prevented intensification of chemotherapy for her malignancy. The addition of basiliximab promoted gradual improvement of the skin, allowing for chemotherapy intensification and subsequent bone marrow transplantation, after which the eruption resolved completely. This report describes a severe case of FUMHD-like eruption associated with clonal leukemic cells that improved with basiliximab, suggesting anti-CD25 therapy as a novel treatment for ulceronecrotic skin disease in the setting of high interleukin-2 levels.

Successful treatment of pityriasis lichenoides chronica with narrow-band ultraviolet B therapy in a patient with Keratitis-Ichthyosis-Deafness syndrome: a case report.

Keratitis-ichthyosis-deafness (KID) syndrome is a rare genodermatosis causing ichthyosis-like skin lesions, keratitis, and deafness. Herein, we report a patient with this rare syndrome in association with pityriasis lichenoides chronica, which was succesfully treated with narrow-band ultraviolet B phototherapy despite our concerns regarding the increased risk of squamous cell carcinoma, hyperpyrexia, and keratitis.

Febrile ulceronecrotic Mucha-Habermann disease in an 8-year-old boy responding to methotrexate.

BACKGROUND: Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. First described by Degos in 1966, it carries a high morbidity and is potentially fatal. The exact pathogenesis is not clear, but it is proposed to be the result of hypersensitivity reaction to an infection. METHODS: Here we report the case of an 8-year-old boy with FUMHD in the eastern province of Saudi Arabia. RESULTS: The patient presented with pyrexia, fatigue, arthralgia, and worsening of his already existing skin lesions of PLEVA. There was an extensive painful erythematous maculopapular rash, some lesions ulcerated and necrotic, involving the face, trunk, limbs, and flexures. A diagnosis of FUMHD was made based on clinical and histological features. The skin lesions responded extremely well to oral methotrexate, with almost complete healing after 4 weeks. CONCLUSIONS: We describe a rare variant of PLEVA, namely FUMHD, in an 8-year-old boy who showed a remarkably favorable response to methotrexate, as manifested by near total clearance of the skin lesions without scarring or hyperpigmentation.

Febrile ulceronecrotic Mucha-Habermann disease: proposed diagnostic criteria and therapeutic evaluation.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare severe variant of pityriasis lichenoides et varioliformis acuta characterized clinically by aggressive ulceronecrotic skin lesions associated with high fever and histologically by features typical of pityriasis lichenoides et varioliformis acuta. Despite the continuous addition of new case reports, no definite diagnostic criteria have been established, and an optimum treatment is still waiting. Herein, we review the different aspects of this rare entity, including pathogenesis, clinical and histopathological features, differential diagnosis, course, prognosis, and outcome. Different diagnostic and therapeutic challenges associated with FUMHD are also evaluated and discussed. We propose two sets of diagnostic criteria to define the disease more precisely and to avoid missing cases. The first comprises constant clinical and histopathological features that are always present in every case, the combination of which is necessary for diagnosis. The second set includes variable features that may be present in some cases and to which any emerging finding could be added. Although different therapeutic options have been used, there is no optimum therapy for FUMHD, and the disease still represents a therapeutic challenge.

Lichen striatus and pityriasis lichenoides chronica in an 11-year-old girl: An etiologic relationship?

Lichen striatusis a rare linear papulardermatosis that primarily occurs in children. The lesions have a linear distribution following Blaschko's lines. Pityriasis lichenoides is an uncommon benign skin disorder with two major variants: acute and chronic.Herein, we report the case of an 11-year-old girl with concurrent pityriasisli chenoides chronica and lichen striatus, a previously unreported association. Although it remains unclear whether there is an aetiological relationship between the two diseases or whether their coexistence was coincidental in our patient, but some common mechanisms may be involved in the two diseases.

Febrile ulceronecrotic Mucha-Habermann disease: a case with systemic symptoms managed with subcutaneous methotrexate.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare, idiopathic, acquired dermatosis that can affect all ages and ethnic groups. We present a 10-year-old patient with FUMHD associated with arthritis and chronic fatigue, managed with methotrexate. Through our literature review, we also explore treatment protocols for a disease for which internationally standardized management is yet to be formulated.