Hailey-Hailey Disease Responding to Thalidomide
A 30-year-old unmarried male presented with a history of recurrent painful fluid and pus filled lesions followed by painful erosions over the neck, axillae, trunk and groins of 15 years duration exacerbated over the past 10-15 days. The lesions were mildly pruritic and the discharge was foul-smelling. The lesions aggravated on sun exposure with exacerbation in summer season. He gave family history of similar disease in his father, paternal grandmother and paternal uncles. Before coming to us, he was diagnosed as a case of HHD, which was confirmed histopathologically. He had been treated with dapsone following which he developed agranulocytosis and dapsone was discontinued. Several other treatment modalities including oral and topical antibiotics, corticosteroids were tried but resulted in only partial or temporary improvement. His condition had been steadily deteriorating.\nOn examination, he was febrile. Large macerated plaques studded with foul-smelling pus along with erosions, fissures, and crusts were present on the anterior and the lateral aspect of the neck, axillae, back, lower abdomen, pubic area, gluteal cleft, groins, upper medial thighs and scrotum []. Surrounding skin was friable and erythematous. Nikolsky's sign was positive perilesionally. Few types of erosion with slough were seen on the tongue dorsum and throat. Erosions and crusts were seen on the glans with purulent foul smelling discharge in the prepucial sac, sparing rest of the penis. Nails showed longitudinal ridging. The skin biopsy specimen for histopathological reconfirmation of the diagnosis revealed focal suprabasilar clefting, few acantholytic cells, intercellular edema with dilapidated brick wall appearance which confirmed our diagnosis [Figures and ].\nPatient was started on intravenous (IV) betamethasone 4 mg, 8th h which was tapered off with the introduction of 40 mg of oral prednisolone, IV ceftriaxone 1 mg twice daily and later IV piperacillin-tazobactam based on pus culture and sensitivity reports along with a combination of fusidic acid and betamethasone for topical application.\nHe continued to develop new lesions with the deterioration of his general condition and was febrile throughout. In spite of controlling the secondary infection, the vesicular eruptions persisted. Therefore, he was started on oral thalidomide 100 mg three times a day and a combination of betamethasone and gentamycin for topical application. Marked improvement was seen within a week of starting thalidomide [] and hence we discontinued corticosteroid after rapid tapering of its dose and continued treating him with oral thalidomide 100 mg three times a day for one more week, which was then tapered off in 3-4 weeks and maintained on 50 mg daily. After 6 months of receiving 50 mg thalidomide, the drug was stopped in view of no recurrence of the lesions. He has improved well and comes to us for regular follow-up.