A case presentation by Dr/ Rania Alakad describing the clinical and histilogic features of a case presented with Febrile ulcero-necrotic Mucha Habermann disease

11.  PLEVA (the febrile ulceronecrotic
variant).
 Erythema multiforme.
 Varicella infection.

12. Investigations
 CBC leukocytosis ( 12.000)
anemia (10 gm/ dl)
 LFT, RFT normal

17.  IVIGS 2 gm / kg
 Cyclosporine
2.5mg / kg
 The patient was
admitted at
another hospital.
 Stevens Johnson
syndrome

18. After 1 week
 No improvement.
 Leucopenia
 Elevated liver
enzymes.
 Elevated serum
creatinine.
Cyclosporine was
stopped.

21.  FUMHD is a rare and potentially fatal
variant of PLEVA.
 classic PLEVA may precede to fulminant
ulcer-necrotic plaques, associated with
systemic symptoms.

23.  The disease typically
affects children and
adults in the third
decade of life.
 Oral and genital
mucosal surfaces
may be involved.
 MRSA & pseudomonas.

26.  unknown.
 hypersensitivity to an infectious agent as
Epstein-Barr virus, cytomegalovirus and
herpes simplex virus.
 an immune mediated process ( CD8 cells
in dermis & epidermis)

27.  FUMHD is characterized by
Histopathological changes consistent
with PLEVA.
 These findings are not specific.
Conditions as erythema multiforme may
exhibit the same findings.

28.  The extensive loss of skin and soft tissue
is similar to patients with severe burns.
Thus, patients with FUMHD should
receive the same supportive intensive
care as the burn victims.

29. 1. High dose glucocorticoids
2. Methotrexate
3. Cyclosporine
4. IVIGs
5. Erythromycin, Acyclovir, Dapsone.

30.  Systemic Vancomycin
and gentamycin were
prescribed after doing
skin and blood culture.
Pseudomonas
areuginosa

32. Generalized edema.
Hypothermia.
Hypotension ,
tachycardia.
Markedly elevated liver
enzymes.
Acute renal failure
Septic shock
Multiple organ
failure
Death

33.  The mortality rate 15 %, with 9 adults
fatalities in the 62 reported cases. Six
of them died of sepsis.
 No reported child fatalities.
 children have favorable outcomes.
 Pulmonary thromboembolism, sepsis
and hypovolemic shock .
to draw attention to this rare disease as
the diagnosis should be considered in
any patient with rapidly progressive
ulceronecrotic lesions with systemic
symptoms. Early diagnosis can avoid
complications or even fatal outcome.
Our messageMortality with FUMHD