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Correspondence to: Leonard C. Sperling, MD, Uni-

formed Services University of the Health Sciences,
4301 Jones Bridge Rd, Bethesda, MD 20814
1. Ishii N, Kawaguchi H, Takahashi K, Nakajima H. A case
of congenital trichofolliculoma. J Dermatol 1992;19:
2. Happle R. Patterns on the skin: new aspects of their
embryologic and genetic causes [in German]. Hautarzt 2004;
55:960-1, 4-8.
3. Happle R. Dohi memorial lecture: new aspects of cutaneous
mosaicism. J Dermatol 2002;29:681-92.
4. Brandling-Bennett HA, Morel KD. Epidermal nevi. Pediatr Clin
North Am 2010;57:1177-98.
5. Happle R. Nevus sebaceus is a mosaic RASopathy. J Invest
Dermatol 2013;133:597-600.

Subcorneal pustular dermatosis associated

with Mycoplasma pneumoniae infection
To the Editor: Subcorneal pustular dermatosis (SPD)
or Sneddon-Wilkinson disease is a rare, chronic, and
relapsing skin disorder, consisting of a symmetric
sterile pustular eruption that primarily involves trunk
and large skin folds. Flaccid, subcorneal pustules
are half-filled with pus with a hypopyon-like
appearance, containing polymorphonuclear neutro- Fig 1. Subcorneal pustular dermatosis. A, Pustular
phils. The pathophysiology is unknown, but eruption on the trunk, the proximal limbs, and in the
common associations include monoclonal IgA large folds. B, Hypopyon-like accumulation of pus.
paraproteins, inflammatory bowel disease, and
rheumatoid arthritis. We describe a rare association Antinuclear antibodies, serum protein electropho-
of SPD with Mycoplasma pneumoniae infection. resis, plasma immunoglobulin levels, and circulating
A 36-year-old woman, without significant per- lymphocyte flow cytometry produced normal or
sonal or familial medical history (including psoria- negative findings. M pneumoniae IgM and IgG
sis), presented with a rash for the last 5 days. She antibodies were elevated, suggesting recent infec-
reported a dry cough without fever, night sweats, or tion. Throat swab polymerase chain reaction was
other symptoms for the last week. Cutaneous exam- positive for M pneumoniae. Chest x-ray and thoracic-
ination revealed a pustular eruption on the trunk, abdominal-pelvic computed tomography scan
proximal limbs, and large folds (Fig 1, A), consisting revealed unremarkable results. Oral dapsone was
of large pustules with hypopyon-like accumulation started but stopped by the patient in a few days
of pus (Fig 1, B). Face, scalp, palms, soles, nails, because of nausea. Cutaneous lesions disappeared
and mucous membranes were spared. The rest in a few weeks with topical corticosteroids alone. No
of physical examination revealed unremarkable recurrence was noted at 18 months follow-up.
findings. No drug had been introduced before In spite of rapidly favorable outcome without
the eruption. Histologic examination showed a recurrence, the clinical and histologic findings were
subcorneal unilocular pustule without spongiosis consistent with SPD. We believe the acute course of
containing polymorphonuclear neutrophils with the disease may suggest an infectious origin. In our
rare acantholytic keratinocytes. Direct immunofluo- case, the patient did not have pneumonia but a mild
rescence did not show immunoglobulin or comple- upper respiratory tract infection that could have
ment deposition. Bacteriologic, virologic, and gone unnoticed. Skin lesions responded to topical
mycologic cultures of pus produced negative results, corticosteroids alone, suggesting that systemic
as did indirect immunofluorescence. Laboratory tests treatment may not be necessary.
found mildly elevated C-reactive protein. These Two similar cases of subcorneal pustules after
findings were consistent with the diagnosis of SPD. M pneumoniae respiratory infections have been

Open access under CC BY-NC-ND license.


described, by Papini et al1 in an 8-year-old boy and a World Health Organization provisional entity here
by Winnock et al2 in a 43-year-old man. In these 2 referred to as Berti lymphoma, comprises less than
cases, dapsone was prescribed without recurrence. 1% of cutaneous T-cell lymphomas (CTCLs).1
Interestingly, M pneumoniaeeinduced Stevens- Berti lymphoma classically presents as eruptive
Johnson syndrome with subcorneal pustules has hyperkeratotic plaques or nodules with a phase
been described a few times.3-5 However, in our of ulceration and necrosis.2 The epidermis
observation mucous membranes were spared, exhibits epidermotropism of atypical pleomorphic
rendering Stevens-Johnson less plausible. We sug- lymphocytes and occasional necrotic keratinocytes.2
gest that there may be a specific presentation of SPD Dissemination to lung, testis, central nervous system,
of infectious origin, possibly more frequent in young and oral mucosa with sparing of lymph nodes is
patients. Our observation highlights the importance common.3 We present a case of Berti lymphoma that
of looking for M pneumoniae in cases of SPD, even arose following a trial of adalimumab in the setting
without respiratory symptoms, and suggests topical of a long-standing spongiotic and psoriasiform
treatment for a few weeks may be sufficient if the dermatitis clinically suggestive of psoriasis.
eruption is considered secondary to M pneumoniae A 35-year-old African American woman had a
infection. 10-year history of nonspecific dermatitis. In the
year before her death, she was admitted to her local
Florian Lombart, MD, Florie Dhaille, MD, Catherine
hospital several times for shortness of breath
Lok, MD, PhD, and Ali Dadban, MD
and dermatitis and was repeatedly treated with
Department of Dermatology, Amiens University prednisone. Skin biopsies from this period showed
Hospital, France a nonspecific spongiotic and psoriasiform dermatitis.
Immunohistochemical analysis failed to demonstrate
Funding sources: None.
any concerning atypical or CD81 epidermotropic
Conflicts of interest: None declared. infiltrate. She was seen by a local dermatologist
as an outpatient, psoriasis was diagnosed, and a
Correspondence to: Florian Lombart, MD, Department
single loading dose of adalimumab (80 mg) was
of Dermatology, Amiens University Hospital,
administered. She presented 2 days later to her
F-80054 Amiens cedex 1, Amiens, France
local hospital for respiratory decline and rapidly
E-mail: developing cutaneous ulceration.
On transfer to our institutions intensive care unit 6
REFERENCES days later, examination revealed skin ulcers localized
1. Papini M, Cicoletti M, Landucci P. Subcorneal pustular primarily to the trunk as well as serpiginous to
dermatosis and Mycoplasma pneumoniae respiratory infection. arcuate scaly plaques and erosions on the extremities
Acta Derm Venereol 2003;83:387-8. (Fig 1). A biopsy on an ulcer edge revealed an
2. Winnock T, Wang J, Suys E, De Coninck A, Roseeuw D.
epidermotropic infiltrate of atypical CD81 cells
Vesiculopustular eruption associated with Mycoplasma
pneumoniae pneumopathy. Dermatology 1996;192:73-4. consistent with a diagnosis of Berti lymphoma
3. Kim H-S, Kim GM, Kim S-Y. A case of Stevens-Johnson (Fig 2). Atypical cells stained diffusely positive by
syndrome with subcorneal pustules associated with immunohistochemistry for CD3, CD8, granzyme B,
Mycoplasma pneumoniae infection. J Eur Acad Dermatol and TIA-1, with 70% of cells proliferating by Ki-67. A
Venereol 2006;20:1353-5.
subpopulation of atypical cells were CD56 positive.
4. Reichert-Penetrat S, Barbaud A, Antunes A, Borsa-Dorion A,
Vidailhet M, Schmutz JL. An unusual form of Stevens-Johnson Atypical cells were negative for CD4, CD5, CD7,
syndrome with subcorneal pustules associated with CD20, CD30, CD57, CD99, HSV, and TdT. Flow
Mycoplasma pneumoniae infection. Pediatr Dermatol 2000; cytometry of peripheral blood was normal, and
17:202-4. HTLV I/II and EBV were negative. Left upper and
5. Sneddon IB, Wilkinson DS. Subcorneal pustular dermatosis.
lower lung biopsies revealed focal involvement by
Br J Dermatol 1979;100:61-8.
CD81 and CD561 atypical large lymphocytes consistent with pulmonary lymphoma. Respiratory
function deteriorated, and she died 2 months after
Rapid deterioration in a patient with primary Adalimumab, a human tumor necrosis factor
aggressive cutaneous epidermotropic CD81 (TNF)-alpha monoclonal antibody, is indicated to
cytotoxic T-cell (Berti) lymphoma after treat a range of autoimmune diseases including
administration of adalimumab psoriasis.4 Whether TNF-alpha blockade is
To the Editor: Primary aggressive cutaneous associated with development of lymphomas is
epidermotropic CD81 cytotoxic T-cell lymphoma, controversial, but rapid and fatal progression of