PMID- 27368714
OWN - NLM
STAT- MEDLINE
DCOM- 20170626
LR  - 20170626
IS  - 1365-4632 (Electronic)
IS  - 0011-9059 (Linking)
VI  - 55
IP  - 11
DP  - 2016 Nov
TI  - Febrile ulceronecrotic Mucha-Habermann disease in an 8-year-old boy responding to 
      methotrexate.
PG  - 1205-1209
LID - 10.1111/ijd.13323 [doi]
AB  - 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.
CI  - (c) 2016 The International Society of Dermatology.
FAU - Alratrout, Jehad
AU  - Alratrout J
AD  - Department of Dermatology, Qatif Central Hospital, Kingdom of Saudi Arabia.
FAU - Alshammasi, Fatima
AU  - Alshammasi F
AD  - Department of Dermatology, Qatif Central Hospital, Kingdom of Saudi Arabia.
FAU - Ansari, Naseem
AU  - Ansari N
AD  - ExpressMed Laboratories, Kingdom of Bahrain. ansarnas@gmail.com.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - England
TA  - Int J Dermatol
JT  - International journal of dermatology
JID - 0243704
RN  - 0 (Dermatologic Agents)
RN  - YL5FZ2Y5U1 (Methotrexate)
SB  - IM
MH  - Child
MH  - Dermatologic Agents/*therapeutic use
MH  - Fever/etiology
MH  - Humans
MH  - Male
MH  - Methotrexate/*therapeutic use
MH  - Necrosis
MH  - Pityriasis Lichenoides/complications/diagnosis/*drug therapy
MH  - Skin/*pathology
MH  - Skin Ulcer/etiology
EDAT- 2016/07/03 06:00
MHDA- 2017/06/27 06:00
CRDT- 2016/07/03 06:00
PHST- 2015/12/03 00:00 [received]
PHST- 2016/01/16 00:00 [revised]
PHST- 2016/02/03 00:00 [accepted]
PHST- 2016/07/03 06:00 [pubmed]
PHST- 2017/06/27 06:00 [medline]
PHST- 2016/07/03 06:00 [entrez]
AID - 10.1111/ijd.13323 [doi]
PST - ppublish
SO  - Int J Dermatol. 2016 Nov;55(11):1205-1209. doi: 10.1111/ijd.13323.