Year : 2006 | Volume
: 51 | Issue : 4 | Page : 272--274
Subcorneal pustular dermatosis and thymoma: An association or a coincidence?
Anuja Agarwal1, KN Shivaswamy1, Barani Raja1, Devinder M Thappa1, Surendra Kumar Verma2,
1 Department of Dermatology and STD, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry - 605 006, India
Devinder M Thappa
Department of Dermatology and STD, JIPMER, Pondicherry - 605 006
A 36-year-old male came to us with a six months history of a generalized, itchy, pus-filled eruption and a change in voice of five months duration. Healthy looking male on cutaneous examination revealed numerous isolated and grouped vesicles and vesiculopustules ranging from 5-10 mm on both normal and erythematous skin over the face, trunk, buttocks and extremities. Both flaccid and tense vesicles were seen along with some of them coalescing to form circinate or polycyclic patterns with crusting. Most of these lesions showed hypopyon formation. Nikolsky«SQ»s sign and bulla-spread sign were negative. Histopathology and direct immunofluorescence substantiated our clinical diagnosis of subcorneal pustular dermatosis (SCPD). On investigation, he was found to have thymoma. We herewith report an association of SCPD with thymoma in this case report, not yet reported in the literature.
|How to cite this article:|
Agarwal A, Shivaswamy K N, Raja B, Thappa DM, Verma SK. Subcorneal pustular dermatosis and thymoma: An association or a coincidence?.Indian J Dermatol 2006;51:272-274
|How to cite this URL:|
Agarwal A, Shivaswamy K N, Raja B, Thappa DM, Verma SK. Subcorneal pustular dermatosis and thymoma: An association or a coincidence?. Indian J Dermatol [serial online] 2006 [cited 2020 Nov 29 ];51:272-274
Available from: https://www.e-ijd.org/text.asp?2006/51/4/272/30293
Subcorneal pustular dermatosis (SCPD) was first described by Sneddon and Wilkinson in 1956. It is a chronic, benign, relapsing pustular eruption, mainly involving trunk and affecting particularly women over 40 years of age. Usually there are no constitutional symptoms., We herewith report an association of SCPD with thymoma in this case report.
A 36-year-old male came to us with a six months history of a generalized, itchy, pus filled eruption and a change in voice of five months duration. Both isolated and grouped pea-sized pus filled lesions were noticed on both normal and erythematous skin, rupturing in two to three days to form thin crusts and healing with a faint pigmentation. There was no history of preceding infection, drug intake or similar disorder in the family.
Healthy looking male on cutaneous examination revealed numerous isolated and grouped vesicles and vesiculopustules ranging from 5-10 mm on both normal and erythematous skin over the face, trunk [Figure 1], buttocks and extremities. Both flaccid and tense vesicles were seen along with some of them coalescing to form circinate or polycyclic patterns with crusting. Most of these lesions showed hypopyon formation. Nikolsky's sign and bulla-spread sign were negative. The scalp, palms, soles, nails and mucous membranes were normal.
ENT examination was unremarkable except for a left aryepiglottic fold cyst seen on indirect laryngoscopy. General physical and systemic examination was normal.
On the basis of the above clinical findings, a clinical diagnosis of SCPD was made. Tzanck smear from one of the vesicles showed the presence of neutrophils but no acantholytic cells. Histopathological examination from one of the vesiculopustules revealed a subcorneal split filled with neutrophils [Figure 2]. Neither acantholytic cells nor spongiosis was evident. The rest of the epidermis and dermis was normal. Direct immunofluorescence from perilesional skin showed scanty deposits of IgA, IgM, C3 and fibrinogen over BMZ and around blood vessels. Routine laboratory investigations including CBC, liver and renal function tests were normal. Chest roentgenogram revealed well-defined soft tissue opacity in the left para-cardiac region suggestive of a mediastinal mass lesion. CT scan of thorax showed a large, moderately enhancing anterior mediastinal mass abutting left anterior chest wall and great vessels with clear demarcation. Ultrasound guided FNAC from the mass lesion depicted a cluster of spindle cells suggestive of thymoma [Figure 3]. Neostigmine test, electromyogram, RA factor, thyroid profile, urine for Bence Jones proteins, serum electrophoresis, cryoglobulins and antinuclear antibody were negative. With these findings an association of SCPD with thymoma was considered.
The patient was put on dapsone 100 mg daily. Most of the lesions cleared within within weeks of therapy but few new lesions still continued to appear. However, on doubling the dose, new lesions stopped appearing. The thymoma was surgically removed following which temporary exacerbation of skin lesions was followed by subsidence.
SCPD is characterized by flaccid pustules or vesiculopustules, when fully formed the pus accumulates in the lower half of the pustule, leaving clear fluid in the upper half. These lesions, isolated or grouped, are superficial, flaccid and easily ruptured by friction. They have a tendency to coalesce forming bizarre circinate or serpiginous patterns. Usually, the eruption fades with a faint pigmentation over which new waves of pustules arise. The areas most commonly involved are the groins, axillae and submammary regions. Palms and soles are occasionally involved, the face very rarely and the head and mucous membranes never. Our patient had facial involvement in addition to the trunk.
The histopathological hallmark of SCPD is a subcorneal pustule filled with polymorphonuclear leukocytes with only an occasional eosinophil. There is neither acantholysis nor spongiosis. The immunofluorescence studies are usually negative. However, intraepidermal IgA as well as BMZ and blood vessel wall deposits of IgM, IgG and C3 have been reported.,,
SCPD has been reported in association with various disorders such as IgA and IgG gammopathies, pyoderma gangrenosum,, rheumatoid arthritis, inflammatory bowel disease, IgG paraproteinemia, CD30+ anaplastic large-cell lymphoma, non small-cell lung carcinoma, apudoma, hyperthyroidism, aplastic anaemia, multiple sclerosis, SLE and mycoplasma pneumoniae infection., The exact pathogenesis of SCPD is unknown.
Whether or not the coexistence of these conditions reflects common pathogenic mechanisms remains to be clarified. TNF-a is increased in SCPD; it may be responsible, in part, for the activation of neutrophils that is a feature of this condition.,
Thymoma, on the other hand, is known to be associated with various hematological, endocrinal, connective tissue and cutaneous disorders such as mucocutaneous candidiasis, alopecia areata, lichen planus and bullous dermatoses (pemphigus vulgaris, pemphigus erythematosus, pemphigus foliaceous)., Ours is a case of spindle cell thymoma associated with SCPD. To the best of our knowledge, this is the first report of such an association. This new observation enlarges the spectrum of diseases associated with SCPD.
|1||Sneddon IB, Wilkinson DS. Subcorneal pustular dermatosis. Br J Dermatol 1979;100:61-8.|
|2||Wojnarowska F, Eady RA, Burge SE. Bullous eruptions. In : Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Rook/Wilkinson/Ebling Textbook of Dermatology, 6th ed. Oxford-Blackwell Science: London; 1998. p. 1817-97.|
|3||Weedon D. Skin Pathology, 2nd ed. Churchill-Livingstone: London; 2002. p. 129-91.|
|4||Sanchez NP, Perry HO, Muller SA, Winkelmann RK. Subcorneal pustular dermatosis and pustular psoriasis: A clinicopathologic correlation. Arch Dermatol 1983;119:715-21. |
|5||Venning VA, Ryan TJ. Subcorneal pustular dermatosis followed by pyoderma gangrenosum. Br J Dermatol 1986;115:117-8.|
|6||Marsden JR, Millard LG. Pyoderma gangrenosum, subcorneal pustular dermatosis and IgA paraproteinaemia. Br J Dermatol 1986;114:125-9.|
|7||Honigsmann H, Trautinger F, Wolff K. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease). In : Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors, Fitzpatrick's Dermatology in General Medicine. 6th ed. McGraw-Hill: New York; 2003. p. 625-7.|
|8||Grob JJ, Mege JL, Capo C, Jancovicci E, Fournerie JR, Bongrand P, et al . Role of tumour necrosis factor -alpha in Sneddon-Wilkinson subcorneal pustular dermatosis. J Am Acad Dermatol 1991;25:944-7.|
|9||Rosenow EC 3rd, Hurley BT. Disorders of the thymus: A review. Arch Intern Med 1984;144:763-70.|
|10||Gibson LE, Muller SA. Dermatologic disorders in patients with thymoma. Acta Derm Venereol (Stockh) 1987;67:351-6.|