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e-meducation.org
Multiple skin lesions
Sunday, 31 October 2004
A 56-year-old white male was admitted to the hospital due to multiple skin lesions. In addition, he complained for dysuria
and hematuria within the last month prior to his admission, with clinical characteristics compatible with urethritis or
prostatitis. One month prior to his admission, pruritic skin lesions appeared, first on his back, and then spread to upper
extremities trunk and finally to the lower extremities. His primary physician treated him with steroids per os but no
improvement was achieved. During the last days prior to his admission the patient noticed an increase in the number of
skin lesions, as well as seropurulent discharge from several of them. The patient did not report arthralgias, anorexia, or
loss of weight. His past medical history included an idiopathic thrombocytopenic purpura episode 8 years ago, a
cytomegalovirus "mononucleosis like" infection 1 year ago, and a urinary truck infection (not otherwise specified) six
months ago.
Physical examination revealed multiple skin lesions with diameter ranging from 0,5 cm to 5 cm (Figures 1 and 2). Some
lesions had a central crust, while others an erythematous halo. The lesions were in different shapes vesicles and
pustules, coalescing in some areas. In some of the lesions, seropurulent discharge was present. No lymphadenopathy
was found. The rest of the physical examination was normal.
A 56-year-old white male was admitted to the hospital due to multiple skin lesions. In addition, he complained for dysuria
and hematuria within the last month prior to his admission, with clinical characteristics compatible with urethritis or
prostatitis. One month prior to his admission, pruritic skin lesions appeared, first on his back, and then spread to upper
extremities trunk and finally to the lower extremities. His primary physician treated him with steroids per os but no
improvement was achieved. During the last days prior to his admission the patient noticed an increase in the number of
skin lesions, as well as seropurulent discharge from several of them. The patient did not report arthralgias, anorexia, or
loss of weight. His past medical history included an idiopathic thrombocytopenic purpura episode 8 years ago, a
cytomegalovirus "mononucleosis like" infection 1 year ago, and a urinary truck infection (not otherwise specified) six
months ago.
Physical examination revealed multiple skin lesions with diameter ranging from 0,5 cm to 5 cm (Figures 1 and 2). Some
lesions had a central crust, while others an erythematous halo. The lesions were in different shapes vesicles and
pustules, coalescing in some areas. In some of the lesions, seropurulent discharge was present. No lymphadenopathy
was found. The rest of the physical examination was normal.
Differential diagnosis
The main differential diagnosis of the disease includes pemphigus foliaceous, pustular psoriasis, acute generalized
exanthematous pustulosis, dermatitis herpetiformis, necrolytic migratory erythema, bacterial impetigo, dermaphytosis,
and subcorneal postural dermatosis.
Diagnosis
Subcorneal pustular dermatosis (SPD). Also known as Sneddon and Wilkinson dermatosis.
Biopsy of the removed skin lesion revealed subcorneal pustules, with a neutrophilic infiltrate and minimal spongiosis. The
pathologist reported that the picture was very suggestive of a pustular dermatopathy, strongly resempling subcorneal
pustular dermatosis. In addition, cultures were negative.
Diagnosis in our patient was, partially, made by exclusion of other diseases with similar manifestations. Specifically, his
negative past medical history, his afebrile, non-systematic, clinical course, the gross morphology and the localization of
the skin lesions were against pustular psoriasis, necrolytic migratory erythema, acute exanthematous pustulosis, and
dermatitis herpetiformis. In addition, histology findings were against pemphigus foliaceous, pustular psoriasis, acute
exanthematous pustulosis, and dermatitis herpetiformis. Finally, the repeated sterile cultures from specimens of lesions
were against bacterial impetigo and dermatophytosis.
Therapy
Treatment with dapsone -the drug of choice for this condition- was considered. However, his skin condition improved
dramatically within the first week after his first biopsy, while we were planning for a second biopsy. At that period the
patient was receiving treatment with azithromycin 250 mg per day by month (for a total of 7 days) for the management of
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possible non-specific urethritis. In addition, he also received local treatment with steroid plus keratolytic agent
(flumetasone pivalate 0.02% + salicylic acid 3%) twice daily, as an empiric regimen.
Useful remarks
- Subcorneal pustular dermatosis (SPD) is a rare, chronic, relapsing, pustular dermatosis that usually develops in
women over 40 years of age. Eruption is variably pruritic. Characteristically, the lesions spare the mucous membranes
and the face. In the contrary, there is a predilection for the axillae, inguinal and mammary folds, as well as for the flexor
surfaces.
- IgA monoclonal gammopathy, IgA multiple myeloma and, less oftern, IgG multiple myeloma have all been reported to
be related to SPD 1,2.
- Therapy of SPD is not uniformly successful. Dapsone, 50-100 mg per os once a day long periods, is considered to be
the most effective therapy. Other commonly used regimens include colchicine, retinoids, corticosteroids (both systematic
and potent local), ketoconazole, sulfapyridine and sulfamethoxypyridazine, all with different rates of success2.
Reference list
- Atukorala DN, Joshi RK, Abanmi A, et al. Subcorneal pustular dermatosis and IgA myeloma. Dermatology
1993;187:124-6.
- Reed J, Wilkinson J. Subcorneal pustular dermatosis. Clinical Dermatology 2000;18:301-13.
Acknowledgements
- This case was prepared for our website by I. Bliziotis, MD.
- A full version of this case and a review of the relevant literature were accepted for publication in the Journal of Infection
[Bliziotis I, Rafailidis P, Vergidis P, Falagas ME. Regression of subcorneal pustular dermatosis lesions with azithromycin.
Journal of Infection, 2004 (in print)].
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