A case presentation by Dr/ Rania Alakad describing the clinical and histilogic features of a case presented with Febrile ulcero-necrotic Mucha Habermann disease
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.
19.
20.
21. FUMHD is a rare and potentially fatal
variant of PLEVA.
classic PLEVA may precede to fulminant
ulcer-necrotic plaques, associated with
systemic symptoms.
22.
23. The disease typically
affects children and
adults in the third
decade of life.
Oral and genital
mucosal surfaces
may be involved.
MRSA & pseudomonas.
24.
25.
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.
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
Editor's Notes
A nine year old male boy was referred to our clinic complaining of 1 month history of skin eruption that started suddenly on the abdomen then spread to involve the limbs & genitals. The skin lesions were associated with burning sensation that became markedly painful. They were also associated with low grade fever and generalized malaise.
The patient received systemic corticosteroids with no response
Skin examination shows lesions at different stages of evolution including multiple erythematous papules and plaques. Some plaques show ulceronecrotic centers with hemorrhagic crusts on top. Tense bullae
We noticed that the lesions on the trunk were arranged in a varioliform pattern
Similar lesions on the back
The hemorrhagic necrotic plaques had flexural accentuation on the axilla
The groins
On the neck
ulceration at the penile region with yellowish crusts.
No clinical signs of systemic affection
Histopathological examination shows focal full thickness epidermal necrosis, perivascular lymphocytic infiltration extending to the deep dermis with RBCS extravasation
No atypical cells were found
Another view showing the necrotic area and perivascular infiltrate
Close up view of the epidermis showing exocytosis, Keratinocytes necrosis and vacuolar interface changes
The lesions showed progressive ulceration, necrosis and gangrene particularly in the intertriginous areas with penile necrosis.
All lesions showed severe secondary infection.
The progression of the necrotic lesions, unresponsiveness to therapy, marked superadded infection
& proper clinic pathological evaluation of the skin biopsy, our final diagnosis is
Cytotoxic attack to altered epidermal ags
Unfortunately within the next 48 hours the patient showed very rapid deterioration of the general condition and the skin lesions that showed severe secondary infection .