A Diagnostically Challenging Case of Febrile Ulceronecrotic Mucha-Habermann Disease in an Adult Female Successfully Treated with Methotrexate and Cyclosporine

Abstract

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.

A Fatal Case of Febrile Ulceronecrotic Mucha–Habermann Disease in a 10-Year-Old Boy

“…[ 4 ] Being a very rare disease, there is inconsistency and scarcity of literature on its therapeutics. There are few encouraging reports of methotrexate, but the numbers are very limited and most of these cases did not have severe FUMHD[ 5 6 ] unlike our patient. We gave preference to methyl prednisolone pulse, thinking about extensive irritant contact dermatitis following application of neem and turmeric paste.…”

Mortality risk factors in febrile ulceronecrotic Mucha- Habermann disease: a systematic review of therapeutic outcomes and complications

“…Several case reports demonstrate the potential for initial misdiagnosis as other conditions, including: Stevens-Johnson Syndrome (SJS), 8 presumed pustular psoriasis, 47 Kawasaki disease, 62 and most commonly chickenpox. 9 , 10 Although FUMHD can be managed with similar treatments to other conditions such as SJS and Kawasaki disease, accurate diagnosis allows for more targeted management which may improve treatment outcomes.…”

Febrile ulceronecrotic Mucha-Habermann disease - a case and treatment review

“…In a recent study, it was estimated that 17.6% of FUMHD cases had a potential trigger identified, most commonly infectious in etiology including mycoplasma [10], varicella zoster virus [14], parvovirus B19 [15], Epstein-Barr virus [16][17][18], herpes simplex virus 2 [19], cytomegalovirus [20], adenovirus type II [21], HIV, and human herpesvirus 7 [22]. Other potential non-infectious triggers include measles vaccine [23], spider bite [24], and drugs such as levamisole-adulterated cocaine [25], tegafur [26], and intravenous vitamin infusion [27]. Other studies suggested that FUMHD might be a variant of cutaneous T-cell lymphoma, owing to the observation of monoclonal T cells in these patients, which also correlated with a higher mortality [28]; this subject remains controversial.…”

“…Clinically, FUMHD is a febrile dermatosis and may initially mimic several other conditions. The extensive list of conditions in the differential diagnosis includes Steven-Johnson syndrome [30], Kawasaki syndrome [31], hemorrhagic chickenpox infection, disseminated herpes zoster, coxsackievirus infection, syphilis, vasculitis, pyoderma gangrenosum, presumed pustular psoriasis [27], guttate psoriasis, lymphoid papulosis, hemorrhagic pitytiasis rosea, bullous erythema multiforme, lichen planus, pemphigus vulgaris or paraneoplasic pemphigus, ecthyma gangrenosum, necrotic folliculitis, Gianotti-Crosti syndrome, erythema nodosum leprosum, blastomycosis, and malignant conditions involving clonal T cell proliferation [12, [32][33][34][35][36].…”

“…Because FUMHD represents a challenging diagnosis and no unified criterion exists, Nofal et al have proposed a subset of criteria comprised of constant features, which are found in every case of FUMHD and can confirm the diagnosis alone. In addition, variable features were identified to help ensure that cases of FUMHD are not missed (Table 1), [12,27]. Skin biopsy of a nonulcerated lesion of FUMHD…”