Department of Internal Medicine,
He had been treated with clobe-
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Federico BARDAZZI on his intertriginous area.
Geriatrics and Nephrology, Valentina Angela
Division of Dermatology, ANTONUCCI
University of Bologna, Aurora Maria
Via Massarenti 1, ALESSANDRINI
40138 Bologna, Italy Carlotta BARALDI
<aurora.alessandrini@alice.it> Vera TENGATTINI
Annalisa PATRIZI
1. Baris D, Zahm SH. Epidemiology of lymphomas. Curr Opin Oncol
2000; 12: 383-94.
2. Gelfand JM, Shin DB, Neimann AL, Wang X, Margolis DJ, Troxel
AB. The risk of lymphoma in patients with psoriasis. J Invest Dermatol
2006; 126: 2194-201.
3. Gattu S, Becker EM, Koo JY. A rare case of non-Hodgkins B-cell
lymphoma in a psoriatic patient: a case report and literature review. J
Drugs Dermatol 2010; 9: 1277-81.
4. Ekström K, Hjalgrim H, Brandt L, et al. Risk of malignant lymphomas
in patients with rheumatoid arthritis and in their first-degree relatives.
Arthritis Rheum 2003; 48: 963-70.
5. Wong AK, Kerkoutian S, Said J, Rashidi H, Pullarkat ST. Risk of
lymphoma in patients receiving antitumor necrosis factor therapy: a
meta-analysis of published randomized controlled studies. Clin Rheu-
matol 2012; 31: 631-6.
6. Girolomoni G, Altomare G, Ayala F, et al. Safety of anti-TNF␣
agents in the treatment of psoriasis and psoriatic arthritis. Immuno-
pharmacol Immunotoxicol 2012; 34: 548-60.
7. Théophile H, Schaeverbeke T, Miremont-Salamé G, et al. Sources of
information on lymphoma associated with anti-tumour necrosis factor
agents: comparison of published case reports and cases reported to
the French pharmacovigilance system. Drug Saf 201; 34: 577-85.
8. Girard C, Guillot B, Bessis D. Gastric MALT lymphoma in a patient
receiving infliximab for psoriasis. Br J Dermatol 2008; 159: 497-8.
9. Mahé E, Descamps V, Grossin M, Fraitag S, Crickx B. CD30+ T-
cell lymphoma in a patient with psoriasis treated with cyclosporin and
infliximab. Br J Dermatol 2003; 149: 170-3.
10. Brown SL, Greene MH, Gershon SK, Edwards ET, Braun MM.
Tumor necrosis factor antagonist therapy and lymphoma development:
twenty-six cases reported to the Food and Drug Administration. Arthritis
Rheum 2002; 46: 3151-8.
doi:10.1684/ejd.2013.1925
A familial case of Hailey-Hailey disease
successfully treated with minocycline and
nicotinamide
Hailey-Hailey disease (HHD; familial benign chronic pem-
phigus) is an autosomal dominant, hereditary, blistering
disorder, characterized by recalcitrant erythema and ero-
sions in the intertriginous area. Although it has been
reported that tetracylines and nicotinamide are effective in
some blistering diseases [1], their effects on HHD remain
unknown. Here, we report a familial case of HHD success-
fully treated with minocycline and nicotinamide.
Case 1
A 43-year-old man was referred to our hospital with a 20-
year history of recurrent erythematous plaques and erosions
EJD, vol. 23, n◦ 2, March-April 2013
tasol propionate 0.05% ointment, which resulted in partial
or temporary improvement. On examination, he exhibited
painful erythematous plaques with scales and crusts on his
neck, axillae, and groin accompanied by an abnormal smell
(figure 1A). A skin biopsy from the erythema revealed a
suprabasal cleft, acantholysis with a dilapidated brick wall
appearance and dyskeratotic cells (figure 1C). We diag-
nosed this patient with HHD. Since methicillin-sensitive
Staphylococcus aureus grew in cultures of the erosive lesion
tissues, treatment with intravenous piperacillin and clobeta-
sol propionate 0.05% ointment was initiated. The treatment
cleared the infectious symptoms in two weeks but the HHD
skin lesions remained. Therefore, we discontinued pipera-
cillin and clobetasol propionate treatment and started oral
minocycline (100 mg per day) and nicotinamide (200 mg
per day), which subsequently the controlled skin lesions
(figure 1B). Two weeks later, minocycline was discontinued
because of drug-induced diarrhea and then the patient’s skin
lesions flared up over the next four months. Thus, we res-
tarted the treatment with minocycline (100 mg per day),
which controlled the skin lesions efficiently.
Case 2
A 78-year-old man, the father of the patient (Case 1), was
referred to our hospital. He had a 40-year history of recur-
rent blisters, erythema and erosions on his intertriginous
area, as in Case 1. He had been treated with betamethasone
valerate 0.1% ointment, which resulted in temporary impro-
vement. On visiting our clinic, he exhibited pruritic plaques
with scales and pus in his axillary area. Histopathological
findings demonstrated acanthosis with suprabasal acantho-
lysis in the epidermis. We diagnosed familial HHD. We
prescribed oral minocycline (200 mg per day) and nicoti-
namide (300 mg per day), which promptly improved the
skin lesions, within two weeks. Subsequently, the doses of
minocycline and nicotinamide were gradually tapered off
over a month without recurrence.
A C
B
Figure 1. Clinical and histological findings of Case 1. A,B)
Clinical photographs of the patient before (A) and after (B) the
treatment. C) Histological findings.
265
 Here, we observed a familial case of HHD responding to
oral minocycline and nicotinamide treatment. Topical and
systemic antibiotic agents in combination with topical glu-
cocorticoids are the mainstay of treatment for HHD [2].
Treatment with antibiotic agents should be initiated if colo-
nization is suspected on the HHD lesions [2]. Although
minocycline is known as an antibiotic, its mechanism of
action may include anticollagenase activity, which may
contribute to suppress blistering formation in HHD patients
[3]. Collagenase activity depends on the presence of cal-
cium and zinc, which seem to be chelated by minocycline
[4]. Moreover, both minocycline and nicotinamide are sup-
Copyright © 2017 John Libbey Eurotext. Downloaded by NYU LANGONE MED CTR SCH OF MED HEALTH SCIENCES LIBRARY on 27/04/2017.
posed to inhibit neutrophil and eosinophil chemotaxis and
lymphocyte transformation, which may also contribute to
their anti-inflammatory effect [5]. The mechanism of action
for tetracycline and nicotinamide in combination remains
unknown but this combinational therapy is reported to
inhibit antigen-induced lymphocyte transformation, leu-
kocyte chemotaxis and histamine release [5]. Thus, these
mechanisms are supposed to provide a useful therapeutic
option in managing patients with bullous pemphigoid [5, 6].
In conclusion, our observation raises the possibility that
minocycline and nicotinamide may constitute an effective
alternative therapy in HHD. To the best of our knowledge,
this report is the first to describe an effect of minocycline
and nicotinamide in controlling the skin lesions in HHD.

Disclosure. Financial support: none. Conflict of interest:
none.
1
Department of Dermatology, Kazuna
Yamato Takada Municipal Hospital, HIGASHIMAE1,2
1-1 Isonokitamachi, Yamato Takada, Kio PARK1
Nara 635-0094, Japan Kenji KABASHIMA3
2
Department of Dermatology, Hiroshi TANABE2
Hyogo Prefectural Tsukaguchi
Hospital,
Hyogo, Japan
3
Department of Dermatology,
Kyoto University Graduate School
of Medicine,
Kyoto, Japan
<kiopark2@gmail.com>
1. Chaffing ML, Collison D, Fivenson DP. Treatment of pemphigus and
linear IgA dermatosis with nicotinamide and tetracycline: a review of
13 cases. J Am Acad Dermatol 1993; 28: 998-1000.
2. Warycha M, Patel R, Meehan S, Merola F. Familial benign chro-
nic pemphigus (Hailey-Hailey disease). Dermatol Online J 2009; 15:
8.
3. Humbert P, Treffel P, Chapuis JF, Buchet S, Derancourt C, Agache P.
The tetracyclines in dermatology. J Am Acad Dermatol 1991; 25: 691-
7.
4. White JE. Minocycline for dystrophic epidermolysis bullosa. The
Lancet 1989; 1: 966.
5. Fivenson DP, Breneman DL, Rosen GB, Hersh CS, Cardone S, Muta-
sim D. Nicotinamide and tetracycline therapy of bullous pemphigoid.
Arch Dermatol 1994; 130: 753-8.
6. Loo WJ, Kirtschig G, Wojnarowska F. Minocycline as a therapeutic
option in bullous pemphigoid. Clin Exp Dermatol 2001; 26: 376-9.
doi:10.1684/ejd.2013.1957
266
Congenital lipomatosis of the scalp: the
importance of investigation for intracra-
nial lipoma
Subcutaneous lipomas are frequently encountered in der-
matology and generally need no further work up. However,
congenital lipomatosis on the scalp is an uncommon clinical
presentation. We report the importance of investigation for
intracranial lipomas in an infant with subcutaneous lipomas,
especially on the scalp.
A 3-month-old female patient presented with several cuta-
neous nodules on the scalp. She had had them from birth
and they showed gradual enlargement. On physical exa-
mination, the nodules were variable sized, ranging from
1-3cm in width. They were soft and well-demarcated, with
focal alopecia above the nodules (figure 1A). Under the
impression of congenital alopecia areata and lipomatosis,
we performed a skin biopsy on the lesion. The pathology
showed decreased hair follicles with mild dermal fibrosis
and thickening of the subcutaneous tissue with proliferation
of mature subcutaneous fat cells (figure 1B). Based on case
reports of previous radiologic studies reporting the rele-
vance of possible intracranial components to subcutaneous
lipomas, we carried out imaging studies. Unenhanced brain
CT showed a hypoattenuated mass near the quadrigeminal
cistern. For further work up, the patient underwent brain
MR with vascular reconstruction, which revealed a soli-
tary, 7 mm lipoma in the left portion of the quadrigeminal
cistern, without connection to the scalp lipomas (figure 1C).
The patient had no ocular or vascular anomalies. The patient
is under clinical follow-up.
A
B C
Figure 1. A) Multiple, variable sized soft nodules with focal
alopecia were observed on the vertex of a 3-month-old female
patient. B) Histopathological examination showing decreased
hair follicles with mild dermal fibrosis and thickening of the
subcutaneous tissue with proliferation of mature subcutaneous
fat cells (H&E, ×50). *Inset: horizontal section (H&E, ×100)
C) T1-weighted axial MR image showing subcutaneous lipo-
matosis on the scalp with high signal intensity (Left, white
arrow) and a 7 mm lipoma in the left portion of the quadrige-
minal cistern, without contiguous extension to subcutaneous
component (Right, white arrow).
EJD, vol. 23, n◦ 2, March-April 2013